Renovascular hypertension associated with a pelvic kidney and multicystic dysplasia

We report on a 9-year-old boy with severe hypertension due to a variant of fibromuscular dysplasia of the renal artery supplying a functioning pelvic kidney. The other kidney proved to be a nonfunctioning multicystic remnant and several other congenital abnormalities of the genitourinary tract were present. Corrective vascular surgery was followed by normalization of arterial pressure and significant reduction of plasma renin activity. This is the first case report of renovascular hypertension due to renal artery stenosis in a single functioning pelvic kidney.