Between 1980 and 1990 transcaval liver resection with hepatoatrial anastomosis was performed in 17 patients with the Budd-Chiari syndrome. There were two early deaths (early mortality 11.7%). Hepatic function returned to normal and hepatosplenomegaly disappeared in all but two patients with preexisting cirrhosis. All survivors regained normal working capacity after the operation. During an average follow-up of 6 years (7 months to 11 years) there were three late deaths due to progression of the underlying disease. The actuarial 1-, 5-, and 10-year survivals were 82%, 76%, and 57%, respectively. Hepatoatrial anastomosis represents an optimal treatment for patients with the Budd-Chiari syndrome and obstruction of major hepatic veins. Patients with compression of the inferior vena cava, very common in this disease, were treated by simultaneous transcaval stenting. The late results are very satisfactory, with excellent quality of life. With adequate hepatic function, results of hepatoatrial anastomosis are superior to those of liver transplantation, which represents the only alternative for patients with the advanced form of the Budd-Chiari syndrome.