An unusual and extreme case of Klippel-Trenaunay syndrome is presented. The patient has an extensive capillary hemangioma involving the right side of the body, increases in the length and girth of the right lower limb, gross varicosities of the superficial venous system, and complete absence of the deep venous system of the right lower extremity, with suprapubic shunt of the venous blood to the contralateral external iliac vein. Secondary manifestations are increased sweating and compensatory scoliosis.