A 54-year-old woman with intestinal multiple smooth muscle tumors including leiomyosarcoma, epithelioid leiomyoma, and leiomyomas in association with von Recklinghausen's disease is reported. Thirteen years after the excision of an intestinal leiomyosarcoma, another leiomyosarcoma arose in a different area of the jejunum and was also completely resected. Although leiomyomas are occasionally recognized in patients with von Recklinghausen's disease and a gastrointestinal neoplasm, there have been no other reports of leiomyosarcomas. Careful observation of the tumor is necessary and, if a rapid increase in tumor size is recognized, malignant tumor may have arisen and early surgical treatment is required.