Mirizzi syndrome is infrequently associated with fistula formation, particularly a unique biliopleural fistula. The ideal therapeutic strategy remains diverse across guidelines, which usually includes surgical excision to eliminate the source of infection. We reported a case of a 60-year-old jaundiced female with pleural effusion resulting from a biliopleural fistula. Imaging revealed a cystic lesion close to the periphery of the upper right diaphragm, connected to the gallbladder. Following initial pleural drainage, an abdominal exploration was conducted to excise the fistula and remove the gallbladder. A multidisciplinary approach is essential for obtaining optimal outcomes for patients with biliopleural fistula.