Endometrial/ioid stromal tumors comprise a spectrum of mesenchymal neoplasms, ranging from benign to low-grade malignancy to undifferentiated sarcomas, which occur predominantly in the uterus but may rarely originate at extrauterine sites, most commonly in the ovary. These tumors and their morphologic variants are important to recognize as they often cause diagnostic difficulties. This review focuses on the diagnostic criteria and differential diagnosis, including the role of immunohistochemistry.