Introduction/Background Endometrial Stromal Sarcomas (ESSs) are very uncommon malignant tumors, accounting for only 0.2 percent of all uterine malignancies, occurring in premenopausal women. We aim to highlight the clinical behavior and management outcome of this rare entity.

Methodology This study reviewed retrospectively 16 patients with histologically proven low-grade stromal sarcoma (LGESS) at Salah Azaiz Institute of oncology, between 2000 and 2021.

Results The median age was 44. 8 years. The most common presenting symptoms were pelvic pain and abnormal vaginal bleeding. Diagnosis was made through curettage in only three patients, however, two patients were diagnosed after a simple hysterectomy that took place in another department and they were sent to our institution for recurrent disease. Five patients were diagnosed at FIGO stage I. The entire patients underwent surgical treatment. Adjuvant therapy was administrated in eight cases. The median follow-up period was 60 months. Two patients were lost to follow-up. Seven patients had disease recurrence. There was no death due to disease progression.

Conclusion Further research studies viewing LGESS and meta-analyses are needed in order to improve treatment options. An appropriate with early diagnosis and intervention are the keys to improve patient survival.