Two children, 8 and 11 years old, presented with severe hypertension secondary to unilateral and bilateral total occlusion of the renal arteries, respectively. The 11-year-old developed sudden anuria requiring hemodialysis. Successful surgical reconstruction allowed recovery of renal function and normal blood pressure in both patients. Routine blood pressure control in the pediatric patient population, high clinical awareness, and judicious use of arteriography, provide the best chance for early diagnosis of renovascular disease. Surgical revascularization or transluminal angioplasty are the treatment modalities of choice in appropriately selected cases of renal artery stenosis. When total occlusion occurs, retrieval or preservation of renal function can be successfully achieved by direct surgical intervention.