The purpose of this article is to review the available literature for uterine papillary serous carcinoma (UPSC). A literature search was conducted to identify publications on UPSC. The literature on UPSC is composed mainly of retrospective, single-institution reports. Despite these limitations, several recommendations can be made. When UPSC is confirmed on preoperative biopsy, complete surgical staging should be performed. Although whole abdominal radiotherapy has a limited role in early-stage UPSC, there may be a role for postoperative chemotherapy in early-stage UPSC. In the setting of optimally debulked advanced-stage disease, a combination of radiation and chemotherapy may be indicated. In the setting of recurrent or suboptimally debulked advanced disease, a platinum-based regimen is recommended. Although comprising a minority of the women with endometrial cancer, women with UPSC do account for a disproportionate percentage of the recurrences. There is a need for clinical trials to determine the optimal therapy for this cohort of patients.