We report a 30-year-old woman with pleuropericarditis, cardiac tamponade, and disseminated intravascular coagulation complicating active ulcerative colitis (UC). Other autoimmune diseases were not present. She responded to pulsed steroid therapy and anticoagulant with resolution of the complication and UC. We reviewed the literature and found 27 cases of pleuropericarditis associated with idiopathic inflammatory bowel disease (IBD). It has been reported that pleuropericarditis associated with IBD responds well to nonsteroidal antiinflammatory drugs, as well as steroids. The causes of cardiac involvement in IBD remain unclear, but the pleuropericarditis must be recognized as a potential extraintestinal manifestation of IBD.