Among complications of fibromuscular dysplasia are uncontrolled hypertension, aneurysm and, albeit very rare, renal failure, especially in a single kidney. We report a case of severe stenosis of fibromuscular dysplasia leading to renal failure, in need of temporary hemodialysis in 29-year-old hypertensive woman. When she was 13-year-old, she underwent a left nephrectomy because of the left renal cyst and she was treated with antihypertensive medication for several years. A few day before admission, she complained of oliguria, generalized edema and aggravated dyspnea. On the day of admission, she took emergency hemodialysis. A renal angiogram showed focal irregular stenosis in the right proximal renal artery, compatible with fibromuscular dysplasia; therefore, we performed percutaneous renal artery angioplasty. Following successful renal angioplasty, her urine output increased and creatinine level was normalized.