Klippel-Trenaunay-Weber (KTW) syndrome is a rare vascular malformation including soft-tissue hypertrophy, varicose veins, cutaneous hemangiomas, and arteriovenous fistulas.The majority of reported cases were discovered at birth; only 7 cases have been reported in utero.Varicosities of the umbilical vein in utero involving the extrafetal portion of the cord are rare.Thrombosis of these varicies often re sults in the death of the fetus.No articles, at this time, report a case of KTW associated with an umbilical varix and subsequent thrombosis.The following case describes the sonographic appearance of both these abnormalities.