[A case of Klippel-Trenaunay-Weber syndrome presenting with esophageal and gastric varices bleeding].

Klippel-Trenaunay-Weber syndrome is a congenital vascular disorder consisted of a variety of vascular malformations, enlargement of the involved limb, and varicose veins. We report a case of Klippel-Trenaunay-Weber syndrome presenting portal hypertension and varices bleeding caused by hypoplasia of the portal vein. Portal hypertension was caused by portal vein hypoplasia associated with Klippel-Trenaunay-Weber syndrome.