Peripheral primitive neuroectodermal tumors (pPNETs) of the ovary are rare monophasic teratomas, and fewer than 100 cases have been reported in the literature. pPNETs mainly involve young women during their reproductive age, therefore, accurate diagnosis followed by multimodal treatment should be taken into consideration for fertility preservation. We report a patient with stage IA pPNET of the ovary presenting with acute abdominal pain secondary to torsion that was successfully managed by fertility-sparing surgery and six courses of combination chemotherapy with vincristine, Adriamycin, and cyclophosphamide. She has had a disease-free survival of >3 years. This brief review demonstrates the clinical course of pPNET and summarizes the literature to show that clinical stage at the time of diagnosis is the most important prognostic factor and that the vast majority of recurrences are observed within 10 years.