Congenital bronchobiliary fistual is a rare disease with unclear etiology. An abnormal tract communicates the tracheobronchial junction to a hepatic segment, usually the left lobe. Billous sputum, a positive HIDA(o-Dimethyliminodiacetic acid) scan, and a trification at the level of the carina lead to the diagnosis, which can be confirmed by bronchoscopic contrast injection. We experienced a case of congenital bronchobiliary fistual in a 27-day-old girl. Our case is reported with literature reviews.