A case of congenital bronchobiliary fistula in a three-year old girl is reported. The patient was born after 40 weeks of gestation and referred due to a long history of respiratory tract problems. Bronchoscopy disclosed a third opening at the level of the carina. The opening was cannulated with an X-ray positive catheter, and a fistula crossing the diaphragm and communicating with the left hepatic duct was found. Cholescintigraphic examination (HIDA-scanning) confirmed the presence of the fistula, but found no evidence of abnormalities in the rest of the biliary system. The thoracic portion of the fistula was excised through a right side thoracotomy. The postoperative course was uncomplicated. HIDA-scanning one month after the operation showed normal uptake, and the girl's symptoms had disappeared.