A patient with hypertension is shown to have both a renal artery stenosis due to fibromuscular dysplasia and a hypoplastic contralateral kidney, a condition comparable to that of the one-kidney Goldblatt hypertension. Both blood volume and plasma renin activity were increased. Blood pressure was lowered either by an angiotensin II analog or by captopril. Secretion of excess renin was observed only from the stenotic kidney. A 4-week period of captopril treatment was accompanied by an acute, reversible deterioration of renal function. Transluminal angioplasty corrected the abnormalities in renin and in blood volume and has kept blood pressure and renal function normal for over 2 years.