Eleven of 47 patients (23%) with sustained bile drainage after Kasai's operation for biliary atresia had major variceal hemorrhage. Hemorrhage was esophageal in origin in five children and from the exteriorized conduit in eight. Two patients bled from both sites. The first major hemorrhage occurred at an average patient age of 40.4 months. Clinical manifestations of portal hypertension were poorly correlated with liver functions, however, markedly decreased calibre of the intrahepatic venous radicles was noted by ultrasonography in all patients examined. Primary treatment was by esophageal endosclerosis or by disconnection of portosystemic shunts coincident with stomal closure. Adjunctive splenic embolization was employed in six patients. Recurrent hemorrhage has not been encountered in limited follow-up.