Valeria Tolosa Villarreal,Sebastián Ben,Marcela Segredo,Noelia Hernández,Diego Greif,Fernanda Nozar, Primary Vaginal Leiomyosarcoma: A Rare Case Reported in Uruguay Case Reports in Obstetrics Gynecology and Reproductive 2024 2674-5070 http://dx.doi.org/10.31487/j.CROGR.2024.01.02 https://www.sciencerepository.org/primary-vaginal-leiomyosarcoma_CROGR-2024-1-102 Abstract: Introduction: Primary vaginal sarcomas are very rare tumors and represent approximately 3% of vaginal cancers, and two-thirds are leiomyosarcomas. On this occasion we report the clinical case of a 35-year-old patient assisted by the Gynecology Oncology team of the Gynecology Clinic A, conducting a review of the bibliography to contribute to the management of this entity. Clinical Case: 35-year-old patient with a diagnosis of primary high-grade vaginal leiomyosarcoma, presented with a vaginal mass, treatment was performed in two stages with tumorectomy and subsequently radical C1 hysterectomy, achieving complete resection of the mass, receiving radiation therapy, brachytherapy, and adjuvant chemotherapy. Thirty-one months after treatment, the patient is asymptomatic, with no elements of recurrence. Conclusion: The treatment of these tumors is not well established. Complete surgical excision with wide resection margins is recommended. The use of radiotherapy and adjuvant chemotherapy must be individualized.Keywords: Vaginal leiomyosarcoma, vaginal tumor, high-grade sarcoma