Introduction: Caroli disease is a rare congenital disorder characterized by segmental, nonob-structive dilatation of intrahepatic bile ducts. The term Caroli syndrome is used for the asso-ciation of Caroli disease with congenital hepatic fibrosis. Case Report: A 37 year old woman, a diagnosed case of Caroli syndrome, was admitted to hospital because of fever, cough and sputum. During the clinical examination and imaging, thoracic empyema and lung abscess was seen in the right hemithorax. Also, pneumobilia was seen in abdominal CT scan.

 Conclusion: Tube thoracostomy was done but lung did not expand. Therefore , right thora-cotomy was performed. Intraoperative finding was as fallows :because of biliary tract infec-tion and abccess formation the tracts were fistulated to the lung parenchyma and pleural space. Irrigation and drainage was performed. After 2 weeks the patient was discharged. He was being fallowed up for 1 year after the surgery and no serious problem was observed.