Obstructive biliary disease in childhood is not common, but should be considered in the differential diagnosis of a child with jaundice, abdominal pain, or an abdominal mass. We have reviewed the experience at Vanderbilt Children's Hospital from 1970 to 1985, during which 87 children 18 years of age or younger with biliary tract disease were seen. Twelve patients (14%) had congenital disorders including choledochal stenosis, Caroli's disease, choledochal cyst, teratoma of the common hepatic duct and common bile duct, congenital septate biliary tree, and isolated atresia of the distal common bile duct. Thirty patients had neonatal cholestatic syndromes, and 37 had calculous disease of the gallbladder or extrahepatic bile ducts. Three patients had obstruction of the common bile duct caused by fibrosing pancreatitis. Two had sclerosing cholangitis. Obstruction of the common bile duct was caused by metastatic neoplasm in three patients. We discuss principles of diagnosis and management.