Biliary disease in infants and children is due to congenital abnormalities such as biliary atresia and choledochal cysts, as well as genetic disorders such as hemoglobinopathies and cystic fibrosis. Several new models for the pathogenesis of extrahepatic biliary atresia and congenital intrahepatic bile duct diseases have been proposed during the past year. Liver transplantation is being increasingly recognized as the ultimate treatment for biliary atresia, even after a successful Kasai operation. Roux-en-Y hepaticojejunostomy is the recommended standard operation for choledochal cysts, although several modifications have now been introduced (the anatomy of the pancreaticobiliary junction is thought to be important in the incidence of postoperative complications). Infants and children are increasingly recognized as having asymptomatic cholelithiasis, which often resolves with time. Three additional cases of hepatocellular carcinoma associated with arteriohepatic dysplasia (Alagille syndrome) have also been reported, and the authors suggest yearly screening with hepatic ultrasound examinations as well as monitoring serum α-fetoprotein levels. Ursodeoxycholic acid is a promising new treatment for patients with cystic fibrosis and hepatobiliary disease, and several small clinical trials have been performed.