Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory arterial disease, commonly involving the renal arteries. Here we report a case of a 16-year-old Chinese male who was found to have severe hypertension with proteinuria for 2 years. Computed tomography showed absence of the left kidney and enlargement of the right kidney. Subsequent angiography confirmed the above findings and revealed narrowing of both the upper and lower branches of the right renal artery caused by FMD. These combined lesions are very rare, and individuals affected are at increased risk of renal dysfunction if left untreated. Treatment with percutaneous balloon angioplasty is the first choice in such a patient and usually results in optimal outcomes.