Placental site trophoblastic tumor (PSTT) with uterine arteriovenous malformation (AVM) is a rare and potentially catastrophic occurrence. A high index of suspicion and immunohistochemistry secured the diagnosis. The use of appropriate imaging modalities led to the identification of the extent of the disease. Sequential planned management from neoadjuvant intensive chemotherapy, bilateral uterine artery embolization, and laparotomy, and coordinated among different medical disciplines resulted to a successful definitive treatment. Due to its relatively chemoresistant nature, hysterectomy is the mainstay of treatment. Adjuvant platinum-based intensive chemotherapy has been shown to improve overall survival in patients with metastatic disease and those with poor prognostic factors. This case of PSTT with a typical clinical profile was noteworthy due to the development of a significant AVM, a rare complication of PSTT. This case report included a review of treatment experiences as well as peculiarities that set PSTT apart from the more common gestational trophoblastic diseases.