A 24-year-old man was seen at the hospital because of right lower abdominal pain. With a diagnosis of acute appendicitis and pan-peritonitis, the patient was operated on. During the surgery, a perforated point 0.5cm in size was found in the anti-mesenteric side of the jejunum, 180cm from the Treitz's ligament. On the mesenteric side, the greater omentum and Douglas' pouch with multiple white nodules were found. Pathologically we diagnosed this case as leiomyosarcoma of the jejunum and peritoneal dissemination. No adjuvant chemotherapy was given. He survived for only 4 months after the operation. Leiomyosarcoma of the jejunum with perforation is rare. A total of 19 cases of the disease which have been reported in a recent one decade from 1988 to 1998 are reviewed.