Extraosseous Ewing's sarcoma is a rare entity that involves the lower extremities, paravertebral regions of the spine, retroperitoneum, pelvis, and the chest wall. Involvement of mesentery as this case has been only reported five times in English literature. An eleven-year-old girl was planned for gynaecological surgery for suspected ovarian dysgerminoma. Intraoperatively she was referred to the surgical team since tumor arising from mesentery of the terminal ileum. Complete resection of the tumor with a right hemicolectomy was done. Histopathological assessment of the tumor revealed features of small blue round cell tumor which after an expert immunohistochemical staining reported as extraosseous Ewing sarcoma. She underwent a course of adjuvant chemotherapy and currently well during follow up. Due to the rarity of this tumor, a proper consensus on management has not been outlined. However, complete resection and adjuvant chemotherapy remain a standard therapy which showed an excellent post-operative outcome.