A primitive neuroectodermal tumor (PENT) belongs to the category of a Ewing sarcoma. A PENT of the uterus is rare and has been known to be very aggressive by nature. Owing to the rarity of the tumor, there is no optimal treatment at present. In many cases, after hysterectomy, chemotherapy or radiation therapy has been performed. However, an effective chemotherapy regimen was unclear. In the soft tissue sarcoma area, the chemotherapy approach has recently greatly improved. Vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide (VDC-IE) therapy has improved the survival rate of patients with Ewing sarcoma/PENT. Thus, VDC-IE therapy may be used for a uterine PENT. Here, we report a case of a uterine PENT in a premenopausal woman successfully treated with multimodality treatment including VDC-IE therapy and discuss the optimal chemotherapy for a uterine PENT through a literature review.