A case of bronchobiliary fistula (BBF) was demonstrated in the setting of polycystic kidney and liver disease (PCKLD) by hepatobiliary scintigraphy. High-resolution noncontrast computed tomography through the liver showed a polycystic liver. A calcified cyst adjacent to the dome of the liver appeared to have a fistulous connection with the lung on coronal reconstructions. Hepatobiliary scan confirmed the fistulous connection. In a review of the literature, BBF has been associated with hydatid cyst disease, trauma, postsurgical states, and malignancy. As demonstrated by this case, BBF is difficult to diagnose and requires a high clinical index of suspicion. Hepatobiliary scintigraphy is the imaging modality of choice to confirm BBF, and PCKLD should be added to the list of antecedent etiologies.