A 7-year-old boy with a history of low-risk acute lymphoblastic leukemia developed multiple intussusceptions shortly after the end of maintenance therapy. Explorative laparotomy showed >10 polyps in the small intestine. Histologic examination revealed intestinal smooth muscle sarcomas associated with Epstein-Barr virus. The patient recovered well after partial cuneiform resection of the largest polyps and treatment with sirolimus. This case report indicates that these tumors may arise even after moderate transient immunosuppression and that association with acute lymphoblastic leukemia is possible although rarely described. We discuss the potential benefit of the mTor/Akt signal inhibitors as treatment for these tumors.