Small bowel metastasis from primary bone leiomyosarcoma is very rare. Here we report on a 50-year-old man who presented with general weakness, weight loss (six kg in two months) and intermittent tarry stools for two months. He had undergone an above-knee amputation for left tibia leiomyosarcoma seven years previously. No local recurrence and/or distant metastasis developed during a seven-year period of follow-up. Subsequent imaging study revealed a multilobulated mass in the ileum. He received segmental resection of the small bowel and a multilobulated mass was noted in the submucosal layer of the ileum with mucosa ulceration. His postoperative course was uneventful. Histopathological examination of the resected mass revealed small bowel metastatic leiomyosarcoma. No local recurrence or distant metastases were detected during a six-month follow-up period. To the best of our knowledge, this is the first report of small bowel metastasis from primary bone leiomyosarcoma presenting with lower gastrointestinal bleeding.