The triad of varicose veins, hypertrophy of soft tissue and bone, and cutaneous hemangiomas (The Klippel-Trenaunay syndrome) often results in major malformations. A review of eleven children treated for this condition showed that the overgrowth of the limb was a major problem in management. In three patients the altered hemodynamics resulted in cardiac complications. Venous stripping, excision of the arteriovenous fistulae, and radiation therapy should be avoided.