Antiphospholipid syndrome is characterized by recurrent episodes of arterial and venous thrombosis, spontaneous fetal losses, thrombocytopenia and persistently elevated levels of antiphospholipid antibodies. We experienced a case of Budd-Chiari syndrome in a 32-year old female lupus patient who was presented with left leg edema, ascites and esophageal varix. The clinical and laboratory findings were compatible with the cirteria for systemic lupus erythematosus (SLE) and she was found to have anticardiolipin antibody, thrombocytopenia and prolonged partial thromboplastin time. Initially, she was treated with intravenous heparin and uroki nase and she was followed up with warfarin, baby aspirin and steroids.