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Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder and is essentially a disorder of capillary, venous, and lymphatic malformations. Hematochezia is the most common symptom associated with intestinal hemangiomatosis and remains one of the life-threatening emergencies in KTS. We reported one patient of KTS presented with rectal bleeding and severe anemia who was successfully managed by sphincter-saving operation.