Case report: We report the case of a 74-year old woman who presented with an ulcerated mass of the vagina. Histology of the tumor showed malignant mixed mullerian tumor (MMMT) with squamous and spindle cell stromal components, associated with high-grade vaginal intraepithelial neoplasia (VaIN 3). Immunohistochemical study of the neoplasm revealed that the malignant stromal tumor was a high-grade leiomyosarcoma. Despite the multimodal therapeutic approach, the patient died within 11 months.
Conclusion: The vagina is a rare site of presentation of primary MMMTs and the present case is the second one immunohistochemically confirmed.