A case of the Budd Chiary Syndrome (BCS) in a 43-yr-old man with hydatid cyst (HC) in the liver is reported from Imam Ali Hospital, Kermanshah, western Iran in 2016. This case was complicated by inferior vena cava (IVC) thrombosis, right atrial clot and pulmonary emboli. Compression of IVC was the likely mechanism. Abdominal ultrasonography revealed a huge HC located in segments near IVC and caused compression of IVC. Transthoracic echocardiography (TTE) revealed IVC and right atrium thrombosis, however pulmonary emboli was not documented in TTE but intra operative exploration showed multiple clot in main and left pulmonary artery branch. The patient recovered after open-heart surgery with removal of right atrial, IVC and pulmonary artery emboli. BCS should be looked for routinely in patients with HC of the liver.