Ask-Upmark kidney was originally described as a congenital segmental hypoplasia of the kidney, but recent data suggest it to be a sequela of vesicoureteral reflux or pyelonephritis in early age. This segmental hypoplasia leads to hypertension in young people. This hypertension is treatable with a partial nephrectomy. This reported patient presented with hypertensive urgency in the OPD. On evaluation, he was found to have Ask-Upmark kidney. The patient was managed conservatively. In case of severe and progressive renal damage, surgical resection with or without transplant can be considered. If the disease is nonprogressive with normal kidney function, the patient can be managed with antihypertensive treatment alone. This is one of the rare causes of hypertension in young that should be kept in mind while evaluating such cases.