Objectives The aim of this study is to describe a case of a patient with primary vaginal sarcoma and review of the literature.

Methods A patient with an anatomopathological diagnosis of Leiomyosarcoma of the vagina was included, who consulted the Gynecologic Oncology section of the Italian Hospital in Buenos Aires. A literature search was made through the PUBMED search engine and the MEDLINE database.

Results A 31 years old patient in a context of a gynecological routine control had been diagnosed with a high-grade sarcoma of the vagina. The lesion was on the right side, occupying part of the middle and upper third of the vagina. The cervix was macroscopically normal and respected. A Magnetic resonance reported a 55x35 mm heterogeneous lesion in contact with uterine cervix. The absence of distant disease was confirmed with a PET-CT.A Radical subtotal colpectomy was performed by laparotomical approach. A leiomyosarcoma was diagnosed at the final pathology report.

Conclusions Primary vaginal tumors are rare entities, represent 1–2% of malignant tumors of the female genital tract. The most frequent histological subtype is squamous cell carcinoma. Primary sarcomas represent only 2% of vaginal tumors. The combination of surgery and radiotherapy are valid treatment options. The small size of the analyzed series makes it difficult to standardize therapeutic behaviors. The best treatment strategy must be personalized.