Budd-Chiari syndrome (BCS) is a rare disorder that arises from obstruction of the hepatic venous outflow tract. BCS causes various clinical status from liver cirrhosis and other systemic diseases that are usually fatal. BCS is caused by hypercoagulability, e.g, arising from malignancy, oral contraceptives, and deficiency of protein S or C. It is not rare that BCS often shows venous thrombosis, including in superior vena cava. We performed a cardiac anesthesia for a 44 year old male with BCS and total superior vena cava syndrome (SVCS) due to the hereditary protein S and C deficiency. Surgical relief of the hepatic outflow stenosis was performed during deep hypothermic circulatory arrest. The patient was managed successfully without conventional intraoperative hemodynamic monitoring such as central venous catheterization, pulmonary artery catheterization, or transesophageal echocardiography due to underlying SVCS and the risk of varix bleeding. After weaning of cardiopulmonary bypass, mild acidosis and hypoxia improved slowly in an intensive care unit. Hypercoagulability was controlled by warfarin during the first postoperative day.