Abstract
Endometrial stromal sarcoma rarely occurs as an extrauterine neoplasm and it is even more unlikely to be found in the vagina. To the best of our knowledge, only six cases of primary vaginal endometrial stromal sarcoma without association with endometriosis have been published to this day. We describe a case of a 58-year-old female with a history of vaginal heaviness caused by a mass lesion. After a biopsy was taken, the histopathological findings and immunohistochemical stains were consistent with low-grade endometrial stromal sarcoma. The patient underwent total hysterectomy and bilateral salpingo-oophorectomy with lymph node dissection followed by hormonal therapy. This line of management was heavily based on the treatment guidelines for endometrial stromal sarcoma.