We report herein a case of a girl with renovascular hypertension associated with VATER association. Her plasma renin activity and aldosterone were high. The ultrasonic echogram and renogram revealed a right hypoplastic kidney without function and a normal-sized left kidney with normal function. Renal angiography revealed a small diameter right main renal artery and a normal left main renal artery with segmental stenosis of left branching renal arteries in the middle segment. Selective renal vein sampling indicated that renin secretion was primarily from the left kidney. This is the first report of renovascular hypertension complicated with VATER association.