The case was a 55-year-old woman. She have been pointed out von Recklinghausen's disease for several years. She was referred to our hospital due to multiple abdominal tumor and severe anemia. Enhanced CT examination revealed multiple intraabdominal tumors with central necrosis. The tumors diagnosed mesenchymal tumors associated with von Recklinghausen's disease, and tumor resection was indicated under laparotomy. Tumors were resected together with small and large bowel. The tumor in the pelvic space was resected together with the uterus and right ureter. She was discharged without any postoperative complications at 15 days after the operation. Because immunostaining was positive for CD34, c-kit and DOG1 and Ki-67-positive cells were 18%, the tumors were diagnosed with high-risk GIST for small bowel.