A 19-year-old man with moyamoya disease was diagnosed as having renovascular hypertension, based on stenosis of the proximal portion of the right renal artery with elevated plasma renin activity. Intravascular ultrasound (IVUS) imaging at the renal artery lesion revealed focal narrowing of the renal artery without vascular wall thickening (i.e., coarctation). The coarctation of the renal artery was adequately dilated by stent implantation after suboptimal balloon angioplasty. After the procedure, the patient's hypertension improved. The findings of the present case suggest that IVUS-guided renal angioplasty is an effective therapeutic procedure for correcting coarctation of the renal artery in patients with moyamoya disease.