An endometrial stromal sarcoma (ESS) is an uncommon uterine neoplasm, and its primary occurrence in the intestine as an extrauterine ESS (EESS) is exceedingly rare. We hereby report a primary EESS arising in the sigmoid colon with a review of the literature. A 52-year-old woman presented with bloody stool and underwent a colon fiberscopy, which revealed a fungating mass obstructing the lumen at the distal sigmoid. A laparoscopic low anterior resection was performed, and an umbilicated polypoid mass was identified; on section, it had infiltrated the mesocolic fat and measured 3.8 cm x 2.5 cm. The tumor showed geographic sheets or nests composed of relatively monotonous stromal cells, expansion or infiltration to the proper muscle and mesocolic fat, and extensive lymphovascular invasion and metastasis to regional lymph nodes and the pelvic peritoneum. The tumor cells were strongly and diffusely immunoreactive for CD10, but negative for c-kit, CD34, and Dog1. Two months later, a hysterectomy with a bilateral salpingo-oophorectomy was performed, and no evidence of an ESS was found in the uterus.