A rare case of renal hypertension in a 3-year-old girl caused by segmental renal hypoplasia of vascular origin is presented. Resection of the hypoplastic segment of the kidney permitted successful treatment of the high blood pressure with preservation of renal function. Elevated plasma renin activity in the renal vein of the affected side and biochemical investigations demonstrated that the resected segment of the renal cortex was the source of the renin leading to hypertension by activation of the renin-angiotensin-aldosterone system.
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