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Klippel-Trenaunay-Weber Syndrome with Kasabach-Merritt Coagulopathy and Hydronephrosis. BACKGROUND: Klippel-Trenaunay-Weber Syndrome is a rare syndrome, consisting of vascular malformation of blood and lymph vessels. CASE CHARACTERISTICS: A newborn female with respiratory distress from birth, and having vascular malformation involving left thigh. OBSERVATION: The neonate also had hydronephrosis and developed complication of Kasabach Merritt syndrome. MESSAGE: Urogenital abnormalities can be present in Klippel-Trenaunay-Weber syndrome but hydronephrosis is rare. Mortality is high with development of Kasabach Merritt syndrome.