Herein, we present the case of a 35-year-old man with a previous history of osteosarcoma of his left tibia followed by multiple episodes of bilateral pulmonary metastases. After being clinically free of disease for seven years, the patient developed a 23 cm mass involving his small bowel wall. To our knowledge, this is the largest reported case of metastatic osteosarcoma involving the small intestine. The metastasis was that of a conventional high-grade osteosarcoma with telangiectatic features. The unusual presentation of this metastasis could be secondary to increased long-term survival of patients with osteosarcoma following more advanced modern therapy.
