A 6-month-old boy in whom inflammatory stricture of the extra-hepatic bile ducts was seen, was reported. After hepatic porto-jejunostomy with Roux-en-Y, jaundice subsided completely and hepatic dysfunction was improved dramatically. Histopathological findings of the liver were similar to those seen in congenital biliary atresia, whereas cholangiographic findings characterized by marked enlargement of the intrahepatic bile ducts were different from congenital biliary atresia.
