To outline the appropriate diagnostic methods and therapeutic options for acquired bronchobiliary fistula (BBF). Literature searches were performed in Medline, EMBASE, PHMC and LWW (January 1980-August 2010) using the following keywords: biliobronchial fistula, bronchobiliary fistula, broncho-biliary fistula, biliary-bronchial fistula, tracheobiliary fistula, hepatobronchial fistula, bronchopleural fistula, and biliptysis. Further articles were identified through cross-referencing. Sixty-eight cases were collected and reviewed. BBF secondary to tumors (32.3%, 22/68), including primary tumors (19.1%, 13/68) and hepatic metastases (13.2%, 9/68), shared the largest proportion of all cases. Biliptysis was found in all patients, and other symptoms were respiratory symptoms, such as irritating cough, fever (36/68) and jaundice (20/68). Half of the patients were treated by less-invasive methods such as endoscopic retrograde biliary drainage. Invasive approaches like surgery were used less frequently (41.7%, 28/67). The outcome was good at the end of the follow-up period in 28 cases (range, 2 wk to 72 mo), and the recovery rate was 87.7% (57/65). The clinical diagnosis of BBF can be established by sputum analysis. Careful assessment of this condition is needed before therapeutic procedure. Invasive approaches should be considered only when non-invasive methods failed.