Objective: Ewing sarcoma is a type of neuroectodermal tumors (Ewing family of tumors-EFT) that mostly affect the bone or soft tissue. Primary uterine Ewing sarcoma is extremely rare. Case report: We report a case of a primary uterine Ewing sarcoma in a 46-year-old patient, treated with total abdominal hysterectomy, and bilateral salpingo-oophorectomy and following adjuvant chemotherapy with 6 cycles of vincristine, doxorubicin, and cyclophosphamide, achieving complete remission for one year. Conclusion: Complete resection for EFT is the first choice of treatment, regardless of their origins. Adjuvant chemotherapy or radiotherapy is mandatory if needed. Due to rarity of the disease, this report re-emphasizes the accurate diagnosis and appropriate treatment for these unusual tumor types occurred in female genital organs.
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