Pathologic findings and immunohistochemistry are essential in making a diagnosis of LCNEC of the endometrium. Primary debulking and surgical staging is typically performed, but if a diagnosis of LCNEC can be made preoperatively with immunohistochemistry, surgeons should consider neoadjuvant chemotherapy due to its high grade histology and aggressive course. Otherwise adjuvant chemotherapy is usually given. Even with early stage disease, the prognosis seems poor. Due to the rarity of this aggressive malignancy, more data is needed to establish incidence.