Bronchobiliary fistula (BBF) is a rare condition that results from the communication between the bile ducts and the bronchial tree. It is characterized by the presence of bile in the sputum as pathognomonic symptom, and it is often associated with suspicious pneumonia. The most common causes include infections (e.g. echinococcosis), hepatobiliary surgery, blunt torso traumas, tumors and percutaneous transhepatic procedures. Opinions about BBF treatment are still controversial as it can be treated by both conservative and surgical procedures, while pharmacological treatments are only rarely used. This case report presents a patient who had been diagnosed with chronic BBF of unknown cause, underwent several ineffective conservative procedures and was at last surgically treated.