The ulcerative colitis is a chronic inflammatory bowel disease with an unknown etiology. The major symptoms of ulcerative colitis are diarrhea, abdominal pain and hematochezia. However, arthritis, skin disorders, hepatobiliary inflammation and uveitis are occasionally recognized as systemic complications. Although there are few reports of coexistent pulmonary and inflammatory bowel disease, the lung is not generally considered to be a target organ in ulcerative colitis. We report a patient with ulcerative colitis-related bronchilolitis obliterans organizing pneumonia confirmed by video-assisted thoracoscopic surgery, who responded to corticosteroid therapy.