A case of renovascular hypertension in a patient with a congenitally solitary kidney associated with retrocaval ureter is reported. Aortorenal bypass with autogenous saphenous vein graft and concomitant mobilization of the ureter by division and reanastomosis of the inferior vena cava were performed. Pathogenesis of the stenotic lesion of the renal artery was fibromuscular dysplasia. Postoperative improvement of hypertension was not so good as expected. Soon after administration of nifedipine (20mg/day) was started, blood pressure decreased down to 130/80mmHg and had been well controlled. Problems regarding renovascular hypertension with a solitary kidney and surgical treatment of retrocaval ureter are discussed.