Endometrial stromal sarcomas (ESS) represent approximately 15-25% of all uterine sarcomas and are divided into three types: endometrial stromal nodule, low-grade ESS or endolymphatic stromal myosis, and high-grade ESS. The low-grade ESS is the most common and has a favorable prognosis with 5- and 10-year survival rates of up to 100%. Recurrence is not uncommon and tends to be limited to the pelvis. However, it shows distant metastases after long tumor-free intervals and the most common affected site is the lung, with reported incidence ranging from 7% to 28%. Here we discribe a case of low-grade ESS with pulmonary metastasis with the brief review of literature. This case, 42 year old woman, who had the past history of LAVH (Laparoscopic Assisted Vaginal Hysterectomy) with clinical diagnosis of uterine leiomyoma but proved low-grade ESS by histology was found multiple mass in lung. After pulmonary metastasectomy, she had no recurrence for 10 months after surgery.