Uterine sarcomas are relatively rare tumors of mesodermal origin that constitute 2-6% of uterine malignancies. Uterine sarcomas include endometrial stromal sarcoma (ESS), leiomyosarcoma (LMS), malignant mixed m llerian tumor (MMMT) of both homologous and heterologous type, pure heterologous sarcomas, blood vessel sarcomas, and lymphoma. Endometrial stromal tumors are divided into three types: 1) endometrial stromal nodule, 2) low-grade stromal sarcoma or endolymphatic stromal myosis, and 3) endometrial stromal sarcoma. The low grade endometrial stromal sarcoma is very rare. It shows occasional local recurrence, which might arise from endometrial stroma, from adenomyosis, and rarely from endometriosis. We have experienced a case of low grade endometrial stromal sarcoma of the uterus in a 54-year-old woman, which is presented with a review of literatures.