Placental site trophoblastic tumor (PSTT) is a very rare form of gestational trophoblastic disease (GTD) that occurs mainly in women who have a history of termination of pregnancy. It has different characteristics from other gestational trophoblastic tumors: it grows slowly, secretes low levels of beta-human chorionic gonadotropin (β-hCG), with low metastatic potential. We report a case of PSTT of a 32-year-old patient. Seven months after delivery, the patient presented at our Center with persistence of menorrhagia for at least 1 month. A slightly high level of beta-human chorionic gonadotropin (β-hCG) was observed. TVUS and MRI, an operative hysteroscopy and a laparoscopy were performed. The histological and immunohistochemical findings demonstrated PSTT. Diagnosis of juvenile cystic adenomyoma (JCA) was also added. A total body CT scan was negative for metastases. A total hysterectomy with salpingectomy was performed. We performed a search of relevant studies about PSTT of the last years. A systematic search of Pubmed databases was conducted. Appropriate search terms were constructed by reviewing abstracts, titles and keywords relating to PSTT known to the authors. All articles known to the authors useful to the review were included, comparing with our clinical case. Stages and treatment are related to survival rates, with long term survival expected for stage I low-risk disease after hysterectomy. Our case is a stage I disease with good prognostic factors (patient's age and absence of metastases) and, as described in the literature, a total hysterectomy with salpingectomy was performed.