OBJECTIVES: The management of hepatic venous outflow obstruction, usually known as Budd-Chiari syndrome, remains complex despite a variety of treatments. METHODS: We present the results from 16 patients with Budd-Chiari syndrome who underwent dorsocranial liver resection and direct hepatoatrial anastomosis over a 10-yr period. The inferior caval vein was occluded in 10 patients. RESULTS: Operative mortality was 12.5% (2/16). During a mean follow-up of 7.2 yr, three patients required reoperation, two of whom had veno-occlusive disease. The late mortality was 14% (2/14). Clinical status improved in terms of: abdominal pain, 81 versus 14%; lower limb edema, 56 versus 14%; hematemesis, 19% versus 0%; hepatomegaly, 94 versus 36%; esophageal varices, 56 versus 7%; splenomegaly, 56 versus 21%; and ascites, 87 versus 29%. Mean serum bilirubin (micromol/l) fell from 40.2 to 27.1 (p = 0.005), and serum albumin remained unchanged. A patent hepatoatrial anastomosis was demonstrated in 10/12 survivors. The actuarial survival rate was 74.2% at 5 and 10 yr. CONCLUSIONS: Thus, in patients with outflow obstruction of the major hepatic veins, transcaval dorsocranial liver resection and direct hepatoatrial anastomosis recreate an adequate hepatic runoff. Moreover, this procedure is appropriate for patients with occlusion of the inferior caval vein, obviates or defers the need for liver transplantation, and prevents recurrence of thrombosis.