The Budd-Chiari syndrome is an uncommon condition in which hepatic venous outflow is obstructed by thrombosis of the major hepatic veins. Many of the cases are idiopathic, but it has been described in association with vena caval webs, abdominal trauma, retroperitoneal neoplasms, and hypercoagulable states. A patient with systemic lupus erythematosus (SLE) who developed the Budd-Chiari syndrome with inferior vena cava thrombosis led to a review of the possible association between SLE and the Budd-Chiari syndrome. The therapy of the Budd-Chiari syndrome with associated vena cava thrombosis is also discussed.