Leiomyosarcoma is rare in ileal location. We report the case of a 61years old female patient presenting with ileal leiomyosarcoma occurring at 14years after a uterine carcinoma treated by radiotherapy. The ileal tumor was treated by surgical resection. This tumor was peculiar by the macroscopic polypoid appearance and by expression of PDGFRA protein together with muscle differentiation proteins: smooth muscle actin, desmin and h-caldesmon. Lymph node necrotizing granuloma diagnosis on the surgical resection specimen lead to the diagnosis of tuberculosis and the patient was treated accordingly. At 3years after the diagnosis, the patient was well, without recurrence or metastases. In conclusion, we report the case of a patient diagnosed with ileal leiomyosarcoma occurring 14years after adjuvant radiotherapy for uterine carcinoma. Analysis of the intestinal resection specimen lead to the diagnosis of associated tuberculosis. Moreover, the leiomyosarcoma was peculiar by PDGFRA expression, feature which might be of clinical relevance since the treatment options in radioinduced tumors associated with other conditions are limited.