A retrospective review of 30 patients with documented Budd-Chiari syndrome disclosed an overall mortality of 57%. Medical treatment alone was associated with an 86% mortality; hepatic failure was the most common cause of death. Mortality was 31% overall for the surgical group, but there were long-term survivors among patients undergoing portacaval shunting. From this series, no single surgical procedure was found to be clearly superior. Surgical treatment with a side-to-side portacaval shunt seems to be the preferred operation when it can be performed. Surgical intervention should proceed soon after the diagnosis is made, lest extension of thrombus occur. Medical therapy most often is ineffective.