Uterine sarcomas have rarely been diagnosed after uterine artery embolization. It remains unclear whether the diagnostic work-up is required prior to such embolization to prevent a missed diagnosis of sarcomas and a delay in providing definitive treatment. Because of the rarity and heterogeneity of endometrial stromal neoplasms, little is known about their epidemiology, pathogenesis, and molecular pathology. The authors report a case of low-grade endometrial stromal sarcoma (ESS) diagnosed after uterine fibroid embolization. Although they performed laparoscopic biopsy of the rapidly growing uterine mass, they could not detect the ESS. Although rare, ESS should be considered in the differential diagnosis of uterine fibroid enlargement. It is essential to assess the risk of malignancy by taking into account the patient's clinical symptoms, results of the physical exam, and imaging findings prior to uterine artery embolization. Pathologic diagnosis should include an adequate biopsy sample and the use of molecular genetic testing.