Background: Extrauterine endometrial stromal sarcoma (ESS) is a rare neoplasm. Little is known about its pathophysiology or best treatment approach. Case: We are describing a case of extrauterine ESS in a 70-year-old woman on hormone replacement therapy and with a history of endometriosis. We also present a brief review of the literature on ESS and its relationship to endometriosis and hormonal therapy. Conclusions: Complete resection should remain the treatment of choice for ESS. Unresectable or metastatic low-grade ESS may respond well to progestin therapy, but outcomes of high-grade ESS tend to be poor.
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