Klippel-Trenaunay Weber syndrome is a congenital vascular disease of unknown cause, in which the blood and/or lymphatic vessels do not form correctly. It is characterized by a triad of symptoms: port wine stains that cover one or more limbs, congenital vascular anomalies, usually varicose veins, absence or duplication of a venous structure, malformation or hypertrophy. Objective: To make an update on the Klippel-Trenaunay Weber Syndrome.