AbstractSeven patients demonstrating the difficulties in diagnosis and management of hepatic vein occlusion are presented. The syndrome may present in an acute form with upper abdominal pain, abdominal swelling, ascites and tender hepatomegaly or in a chronic form, mimicking cirrhotic ascites. The clinical features, predisposing factors, liver scan and liver biopsy may all suggest the condition, but hepatic venography is essential for diagnosis and as a preliminary to treatment. It is suggested that early side to side portacaval anastomosis is the current treatment of choice.