Congenital bronchobiliary fistula (CBBF) is an extremely rare anomaly with myriad presentations that often include common bile duct abnormalities. Traditionally, bronchoscopy and bronchography have been used to establish the diagnosis. A full-term neonate with pneumonia and bilious secretions was diagnosed as having CBBF by means of a HIDA scan. Successful repair consisted of a right thoracotomy, high ligation of the fistula via an extrapleural approach, and cholecystography to confirm biliary drainage into the duodenum. The HIDA scan is a safe and efficient means to diagnose CBBF. When used in combination with surgical contrast studies, it provides clear delineation of all variations of this abnormality, enabling one-stage correction.