Objective: To explore the clinicopathologic feature and differential diagnosis of small cell carcinoma (SCC) of the ureter. Methods: The clinical, histopathological and immunohistochemical features of one case of SCC of the ureter were analyzed and the literatures were reviewed. Results: A 70-year-old female presented with a left flank pain 22 days. CT showed the left ureter expansion, and the lower ureter slightly hyperattenuation. The imaging diagnosis did not exclude the possibility of ureteral tumors. Intraoperative frozen section analysis suggested small round cell tumor. The patient underwent left nephroureterectomy. Pathological examination showed that the tumor tissue consisted of small, round or oval shaped cells, with a prominent nucleus, scant cytoplasm and granular chromatin. A high mitotic index may be observed. Tumor cells expressing Syn, ChA and NSE in immunohistochemical staining. Conclusion: SCC of ureter is a relatively rare malignant tumor with high invasiveness and poor prognosis. The pathological diagnosis depends on pathological and immunohistochemical examination. Neuroendocrine markers positive is one of the important reference. The differential diagnoses included poorly differentiated UC, plasmacytoid carcinoma, malignant lymphoma, lymphoepithelioma like carcinoma, or primitive neuroectodermal tumor. The multimodal therapy of these tumors included surgery, chemotherapy and radiation.