Ewing sarcoma/primary neuroectodermal tumors are rare, invasive, and small round blue cell tumors. There are few reports of its occurrence in the urinary system. Here, we present the first middle-aged female patient whose Ewing sarcoma primary site was in the ureter. The main clinical manifestation was intermittent hematuria. She was in good health after complete surgical resection and adjuvant radiotherapy. To date, there has been no recurrence or metastasis. Accurate early diagnosis and appropriate treatment can help prolong survival. 18F-fluorodeoxyglucose positron emission tomography/computed tomography is expected to be an effective means of evaluating treatment effects and detecting metastasis and recurrence. In this article, besides introducing a case of Ewing sarcoma/primitive neuroectodermal tumor of the ureter,we review the literature to discuss the current status of diagnosis and treatment.