Patients with inflammatory bowel disease are at increased risk for thromboembolic complications. Its prevalence rate ranges from 1% to 7% in clinical studies and reaches 39% in autopsy series. The cause of thrombotic complications in inflammatory bowel disease is generally considered to be associated with hypercoagulability. We experienced a case of ulcerative colitis associated with Budd-Chiari syndrome and superior mesenteric vein thrombosis. This rare complication of ulcerative colitis was successfully managed by conventional treatment for ulcerative colitis and anticoagulation therapy.