Primary Ewing sarcoma of the uterine cervix is an exceptionally rare and aggressive malignancy, with only 34 cases reported in the literature. Early recognition and prompt multimodal treatment are essential due to its poor prognosis. We report a case involving a 32-year-old multiparous woman who presented with foul-smelling vaginal discharge and irregular bleeding. Clinical examination revealed a 6×6 cm friable cervical mass extending into the upper vagina. Imaging demonstrated an FDG-avid cervical mass with regional lymphadenopathy. Histopathological analysis, supported by immunohistochemistry and fluorescence in situ hybridization, confirmed Ewing sarcoma with EWSR1 gene rearrangement. The patient received neoadjuvant chemotherapy with VDC (vincristine, doxorubicin, cyclophosphamide) and PIE (cisplatin, ifosfamide, etoposide) regimens, followed by surgical resection, adjuvant chemotherapy, and pelvic radiotherapy. Although initial disease control was achieved, the patient experienced disease recurrence with distant metastases after a 7-month disease-free interval and succumbed to the illness 9 months after diagnosis. This case underscores the importance of including Ewing sarcoma in the differential diagnosis of rapidly growing cervical tumors, particularly in young women. A multidisciplinary approach involving early molecular diagnostics and aggressive combined therapy is critical to improving clinical outcomes in such rare and aggressive cases.