Congenital tracheobiliary and bronchobiliary fistulae are rare malformations in which patent communications exist between the respiratory system and biliary tract, respectively. We present a newborn who was admitted with respiratory distress and bilious tracheal discharge. Investigation revealed a bronchobiliary fistula originating from the left main bronchus, as well as biliary atresia. Excision of the bronchobiliary fistula was successful and the connection between biliary tract and gastrointestinal system was established by performance of a Roux-en-Y cholecysto-jejunostomy. Diagnostic tools, differential diagnosis, and surgical correction strategies are discussed.