Background: The study aimed to examine all available evidence regarding primary vaginal cancer arising from endometriosis from an evidence-based perspective. Methods: A literature search in Medline and EMBASE databases was conducted. The following key words were employed: ‘vaginal cancer' AND ‘endometriosis malignant transformation' OR ‘endometriosis'. Results: A total of 23 eligible studies were identified and included in the present review providing information for 37 patients. Endometrioid adenocarcinoma (17 cases) was the most common histological subtype followed by endometrial stromal sarcoma (6 cases). The majority of patients received cancer-directed surgery while adjuvant treatment was commonly employed for patients with sarcomas. Conclusion: Vaginal cancer arising from endometriosis is exceedingly rare. The mainstay of treatment is surgical excision. Mean follow-up of patients was relative short. Tumor recurrence was often observed, and the outcome was less favorable for patients with non-epithelial tumors.