SummaryA 28 year old woman with systemic lupus erythematosus presented with hepatomegaly and ascites.Clinical, histological and radiological investigations demonstrated the Budd-Chiari syndrome. The patient had two risk factors which could induce hepatic vein thrombosis: use of oral contraceptives and presence of the lupus anticoagulant. Despite fibrinolytic therapy followed by warfarin and corticosteroid, the patient died of hepatic failure five months after diagnosis. The so-called idiopathic Budd-Chiari syndrome could be due, at least in some patients, to the thrombogenous action of the lupus anticoagulant.