Endometrial stromal tumors are very rare, representing approximately 0.2% of uterine malignancies, having an incidence of one to two from a million of women. The diagnosis cannot be established by imaging, it is histopathological only, often necessitate supplementary immunohistochemistry tests. We report the case of a 27-year-old woman who had an initial diagnosis, in another hospital, of uterine adenomyoma, established by dilatation and uterine curettage and then by subsequently histopathological exam. This diagnosis led to an initial non-oncological surgery, with interannexial total hysterectomy. The establishment of the final histopathological diagnosis of stromal endometrial sarcoma has led to a serious reassessment of the case. Making a review of the literature, we found very few cases of endometrial stromal sarcoma in young women less than 30 years old and we have not identified any clear strategy of treatment. However, from precautionary and considering that may be at risk, even with very few cases reported, the distance metastases can be present, sometimes at large intervals of time, we decided, for oncological safety, reintervention after one month. At the second surgery, it was practiced bilateral salpingo-ovarectomy, cardinal ligaments excision, partial omentectomy, bilateral pelvic lymphadenectomy extended lumbo-aortic and interaortico-cava, sampling biopsy from the inguinal femoral adenopathy and re-excision of the vaginal vault. The evolution was favorable, the patient being follow-up together with the oncologist specialist.