A case of Budd-Chiari syndrome in which direct surgical intervention was successfully performed is reported. A 43-year-old female who had had a history of hepatic coma was pointed out complete obstruction of inferior vena cava (IVC) between the right atrium and diaphragma, associated with hepatic dysfunction and esophageal varices. At operation, the IVC lesion was visualized directly by thoracotomy through midsternal incision and by laparotomy through right hypochondrial oblique incision, with the liver retracted. Under partial extracorporeal circulation with suction of blood from hepatic vein, the IVC was incised, 3cm in length, and membranous tissue causing obstruction was resected. The defect of the IVC wall was repaired with ringed EPTFE patch. Postoperatively, both central venous and portal pressure were decreased, 21 mmHg to 10mmHg and 26cm H₂O to 21cm H₂O, respectively, with good patency of the IVC on venogram. Now the patient is up and well, 11 months after operation. Thus, complete removal of obstruction under direct vision is thought to be important for surgical treatment of Budd-Chiari syndrome.