RVH remains a diagnostic and therapeutic challenge in the pediatric population . Various diseases, including FMD, developmental RAS, neurofibromatosis type 1 (NF-1), Moyamoya disease, vasculitis such as Takayasu’s disease, Alagille syndrome, Marfan syndrome, and Williams syndrome, are associated with pediatric RVH [1, 8]. FMD is an idiopathic angiopathy characterized by non-atherosclerotic and non-inflammatory fibroplastic disease that most commonly affects the renal and carotid arteries, and is the most common cause of pediatric RAS [9, 10]. In general, it is four times more prevalent in females than in males in adults, although the sex distribution in children is equal . FMD may be histologically classified based on the arterial wall layer involved—i.e., medial (70 % of cases), perimedial (15–25 %), and intimal fibrodysplasias (1–2 %) . The intimal type of FMD usually presents with a highly stenotic, usually proximal, localized lesion with post-stenotic dilatation. Angiographic features of the intimal type of FMS are more commonly noted on angiography in children than in adults. Both the medial and perimedial types of FMD manifest as alternating stenosis and aneurysms, such that they appear as a “string of beads” on angiography. However, this classic “string of beads” pattern is not frequently observed on angiography in children [1, 2, 8]. As FMD is often a suspected diagnosis or diagnosed based on exclusion, the distinct clinical features between children and adults should be clearly determined. In particular, the medial type of FMD is less commonly encountered in children. In contrast, the intimal type of FMD is uncommon in adults, but is the most common type in children . Among the three histologic types of FMD, dilation is reported to be more difficult in the intimal type than in the medial or perimedial types. FMD is diagnosed based on the angiographic appearance and following the exclusion of other conditions. In the present case, although the patient could not be diagnosed based on the results of histological examinations, we suspected a diagnosis of intimal-type FMD, based on the patients’ history, laboratory results, angiographic appearance (focal stenosis in the middle of the renal artery with poststenotic dilatation, and no characteristic beaded pattern), and resistance to PTRA. Although the technical success rate of pediatric PTRA is high, the long-term success rate of pediatric PTRA is lower than that in adult; this difference may be due to the presence of a smaller vessel diameter and a more pronounced response to growth factors in immature vasculature . Several imaging modalities such as CCA, Doppler ultrasonography, renal scintigraphy, computed tomography angiography (CTA), and magnetic resonance angiography (MRA) have been used to evaluate the renal artery. CCA is the gold standard for the diagnosis of RAS and is especially useful in the evaluation of branch vessel disease. However, CCA requires arterial catheterization and the use of general anesthesia in young children, and is associated with a small risk of complications. CTA is a non-invasive technique that does not require arterial contrast injection, and reportedly has equivalent accuracy as that of MRA in adult patients . MDCT can provide more detailed information for the three-dimensional visualization of RAS, with a shorter scan time, less respiratory motion artifacts, and high resolution, in a non-invasive manner ; moreover, the associated cost and ionizing radiation exposure is lower than those associated with CCA [3, 12]. This could facilitate the planning of the interventional procedure, and may shorten the catheterization time and reduce the contrast injection dose required. Due to these advantages, CTA is currently used for pre-mapping, before the interventional procedure, in some centers . The current day-to-day medical practice guidelines recommend that FMD should be diagnosed based on the CTA or MRA findings. FMD may also be diagnosed using CCA (the gold standard), in cases where FMD is strongly suspected but the non-invasive test results are inconsistent. During follow-up periods, CTA may also be useful to evaluate the effect of the therapies . At present, PTRA is considered the treatment of choice for RAS as a result of FMD, and has a high technical and clinical success rate [4, 7]. The treatment of RAS with PTRA may be difficult because of arterial wall thinning, friability, and the firm elastic recoil of the intramural lesion in some conditions such as NF-1, William syndrome, or mid-aortic syndrome. In few cases, PTRA may not be able to dilate the RAS, and hence, the success rates may vary from 48 to 90 % [7, 9, 14]. Even if it is not curative, PTRA may play a role in temporarily managing hypertension in younger children prior to surgical repair scheduled during puberty. In the present case, as the hypertension was well controlled with anti-hypertensive medication after initial PTRA, the decision to perform either re-intervention or open surgery was delayed until puberty, when the procedure can be performed with better long-term results, even though the result of initial PTRA was unsatisfactory. CBA is reportedly effective for treating stenotic lesions related to FMD that are resistant to conventional balloon angioplasty, particularly in cases of stenosis related to intimal FMD . At present, cutting balloons are available in a wide variety of diameters and lengths, with a range suitable for the treatment of pediatric lesions, and have improved the technical success rate of PTRA. The complications associated with CBA in the treatment of RAS are similar to those for PTRA, including dissection, rupture, and thrombosis of the renal artery . Although CBA extends the therapeutic potential for resistant RAS to small children, it is not a panacea and clinicians should not expect successful results in all children.