A rare case of von Recklinghausen's disease complicating leiomyosarcoma of the stomach and neurofibroma of the jejunum is reported. A 58-year-old male with a chief complaint of epigastric discomfort was diagnosed as having a malignant submucosal tumor or the stomach by upper gastrointestinal radiography, endoscopy and angiography, and was subjected to surgery. Since a thumb-sized tumor was found in the jejunum during laparotomy, total gastrectomy and partial jejunectomy were performed. Postoperative pathological examination indicated that the tumors were leiomyosarcoma of the stomach and neurofibroma of the jejunum. Cases of von Recklinghausen's disease complicating myogenous tumors are rare. Only 12 cases including the present case have been reported in Japan. The above patient had neurofibroma of the jejunum in addition to the rare complication of von Recklinghausen's disease and leiomyosarcoma of the stomach, and to our knowledge, this is the first case of its kind to be reported in the literature.