Congenital tracheobiliary or bronchobiliary fistula is a rare developmental anomaly with a persistent communication between the biliary system and the trachea or bronchus. We report a case of a congenital tracheobiliary fistula and hypoplastic common hepatic duct associated with hypoplastic left heart syndrome in a 5-day old boy presenting with bilious endotracheal-tube secretions. The tracheobiliary fistula was treated by surgical resection. Subsequent cholangiography demonstrated dilated intrahepatic bile ducts and a residual fistulous tract with cystic proximal stump that were successfully decompressed by transhepatic drainage catheters before corrective biliary surgery.