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BACKGROUND Budd-Chiari syndrome (BCS) is defined as hepatic venous outflow obstruction (HVOO). BCS is an uncommon, life-threatening liver disorder. This study describes the clinical and etiological characteristics in addition to the long-term outcome of BCS in a single referral center in Tehran, Iran. METHODS We reviewed long-term outcome of patients who were diagnosed with BCS between 1989 and 2012 at Shariati Hospital, a tertiary hospital affiliated with Tehran University of Medical Sciences, Tehran, Iran. The diagnosis was confirmed by at least two imaging techniques. A comprehensive analysis of the clinical and paraclinical manifestations, etiology and long-term outcome of the disease was conducted. RESULTS Seventy one patients (43 female) with a diagnosis of Budd-Chiari syndrome were identified during the 22 year period of study. The age were ranged from 17 to 64 years (median: 29 years). We excluded 16 patients because of incomplete information or follow up. The remaining 55 cases were the subjects of this study. Underlying etiologies consisted of congenital thrombophilia factors in 50% (28 cases) which was defined as protein C deficiency (12 cases), protein S deficiency (3 cases), antithrombin deficiency (3 cases) and factor V Leiden mutation (10 cases). Etiology was unknown in 18% (10 cases). Acquired causes of thrombophilia were observed in 25% (14 cases) that consisted of 9 cases of myeloproliferative disease and 5 cases of autoimmune diseases. In 3 cases pregnancy was the only etiology. The main clinical presentations were abdominal pain in 33 (60%), abdominal distention in 21 (38.2%), and jaundice in 10 (18%) cases. The main clinical signs were ascites (76.4%), splenomegaly (34%), hepatomegaly (25.5%) and deep vein thrombosis (1.8%). All 55 patients were treated with anticoagulants (heparin followed by warfarin) and supportive care. Two cases underwent mesocaval shunt surgery, 2 patients required transjugular portosystemic shunt (TIPS) and 5 were referred for liver transplantation. A total of 17 (30%) patients died during 22 years of follow up. CONCLUSION BCS, although uncommon in Iran, is a challenging liver disease with an important burden. Medical therapy that includes anticoagulation seems to be effective in most cases although the prognosis is guarded. In long-term follow up, 40% of cases will need liver transplant or die from end stage liver disease. [ABSTRACT FROM AUTHOR]