Endometrial stromal sarcoma (ESS) is a rare malignant tumors of the uterus. Extrauterine ESS is uncommon tumor that occurs in women over a wide age range. Primary ESSs of the ovary are also rare gynecologic malignancies. The extrauterine location, non-gynecologic symptoms and signs at presentation, and confounding histologic features can pose a diagnostic challenge. Extrauterine ESS combined with other form is particularly rare. The majority of extrauterine ESS is combined with endometriosis. Development of extrauterine ESS form dermoid cyst is extremely rare. We experienced a case of advanced-stage high-grade ESS of the ovary combined with dermoid cyst. We describe a case of a 62-year-old woman, who received debulking operation of adnexae with detected high-grade ESS of the ovary combined with dermoid cyst. The patient was then treated with adjuvant chemotherapy. She has a close observation with no recurrence. In of this case, we aimed to discuss high-grade ESS of the ovary combined with dermoid cyst and a review of the literature.