A rare kind of Gestational trophoblastic Disease (GTD) that is Placental site trophoblastic tumor (PSTT) represents a rare kind of tumor originating from placental area intermediate trophoblasts. In contrast to hydatiform mole, invasive hydatiform mole as well as choriocarcinoma to diagnose PSTT is more complex in view of the absence of particular tumor markers that are specific as well as sensitive. Since most PSTT women show a malignant tendency the best way of treating PSTT primarily is hysterectomy. But most of PSTT cases are young women hence fertility preservation becomes essential to be kept in mind. Since rarely metastases takes place that might even result in death in low percentage PSTT patients, hence it is important to treat them with adequate chemotherapy as per individualization of the case and better to use chemotherapy in FIGO stage II-IV. The biggest hurdle in improving prognosis of PSTT cases is chemotherapy resistance. Thus importance of finding adequate tumor biomarkers in view of various targeting factors like VEGF, TGF-β3 signaling as well as prokineticin signalling so that adequate targeted therapy can get initiated. Other important aspect is utilization of immunotherapy on the basis of programmed death -1 (PD1) antibody like nivolumab might be important. Still need of the hour is to carry on multicentric studies to understand the pathogenesis of this very rare kind of GTD for improving its prognosis.