Epithelioid trophoblastic tumor (ETT) is the rarest type of gestational trophoblastic tumor, first identified in the 1980s and formally recognized in the 1990s. Its histological features resemble an epithelioid somatic carcinoma with tumor cells recapitulating the intermediate trophoblast at the chorionic laeve. The term “atypical choriocarcinoma” was used by Mazur in 1989 to describe persistent lung metastases after chemotherapy in patients with choriocarcinoma, which is thought to represent one of the first documented cases of ETT. Later, in 1993, Silva and colleagues reported similar tumors in the uterus following the evacuation of hydatidiform moles, referring to them as “multiple nodules of intermediate trophoblast.” In 1994, Mazur and Kurman coined the term “epithelioid trophoblastic tumor,” which was formally established in 1998 by Shih and Kurman in their report on 14 cases of ETT, as a distinct entity of trophoblastic neoplasm. This tumor is typically associated with a relatively indolent clinical course, although metastasis can occur, particularly to the lungs. The tumor is characterized by the presence of epithelioid cells, which immunohistochemically express markers such as human chorionic gonadotropin (hCG) and cytokeratin.