A patient with hemolytic uremic syndrome (HUS) developed peripheral gangrene involving all fingers and toes. There was no history of bloody diarrhea. Hypocomplementemia was present, with a serum C3 concentration of 41 mg/dl. Acute renal failure was treated with peritoneal dialysis for 4 months. He received daily fresh-frozen plasma infusions and plasmapheresis on alternate days for ten sessions, followed by once-weekly sessions. He was anuric for 9 weeks. All medial and distal phalanxes became necrotic and were removed surgically. The renal biopsy findings were consistent with HUS. This is the second report of peripheral gangrene during the course of HUS in childhood.