BACKGROUND Various surgical procedures for treating chronic Budd-Chiari syndrome have been established, but none are adequate because of the variation in underlying pathologic vascular changes. METHODS This article presents a 32-year-old patient with a 5 cm long segment of obstruction of the retrohepatic inferior vena cava involving the main hepatic veins with severe portal hypertension. RESULTS After five unsuccessful attempts at percutaneous transluminal angioplasty, simultaneous hepatic venoplasty was conducted with the saphenous vein patch and retrohepatic inferior venacavoplasty by the expanded polytetrafluoroethylene patch with a 3 cm long cuff interposition for suprahepatic reconstruction of the inferior vena cava. Hypothermic preserved liver perfusion after vascular isolation and femoroportoaxillary venovenous bypass with a centrifugal blood pump throughout the anhepatic stage ensured safe operation on the liver and maintained hemodynamics. Early postoperative anticoagulant is recommended. CONCLUSIONS An uneventful postoperative course and a 10-month follow-up showing excellent condition indicated this one-stage simultaneous patch hepatic venoplasty and cavoplasty to be adequate for appropriate correction very of complex obstructive vascular lesions in Budd-Chiari syndrome.