We report the case of a young woman with Budd-Chiari syndrome in whom mesentericoval shunt was first performed, followed by transcaval liver resection and hepatoatrial anatomosis 3 years later. Liver transplantation became necessary 5 years later because of deterioarating liver function with portal hypertension and bleeding. Successful transplantation was performed with atrioatrial anastomosis with help of cardiopulmonary bypass, simplifying considerably the technical procedure and reducing dramatically blood loss.