<b><i>Background:</i></b> Epithelioid Trophoblastic Tumor (ETT) and Placental Site Trophoblastic Tumor (PSTT) are two of the rarest GTNs that share certain features at diagnosis and management. Atypical Placental Site Nodule (APSN) is a relatively new entity considered as a premalignant lesion. <b><i>Objectives and Methods:</i></b> The aim of this review was to summarize the main characteristics of each of these entities, their diagnostic features, and their treatment’s standard of care including fertility-sparing treatments. <b><i>Outcome:</i></b> This study provides a thorough review of ETT, PSTT, and APSN. <b><i>Conclusions:</i></b> The reader will gain an insight view of these rare tumors arising from the intermediate trophoblast.