Background <p>Small cell neuroendocrine carcinoma of the urinary tract (SCC-UT) is an extremely rare genitourinary tumor, which is highly aggressive, prone to local or distant metastasis and has a very poor prognosis.</p> Case presentation <p>This article reports a case of primary small cell neuroendocrine carcinoma of the ureter combined with invasive uroepithelial carcinoma. The patient underwent robot-assisted laparoscopic radical left nephrectomy, full-length ureteral resection, sleeve cystectomy, and pelvic lymph node dissection after exclusion of contraindications to surgery (preoperative assessment confirmed the absence of cardiopulmonary or coagulation abnormalities) and underwent 6 cycles of post operative etoposide/cisplatin adjuvant chemotherapy.</p> Conclusions <p>This case underscores the potential efficacy of multimodal therapy, combining robot-assisted surgical resection with adjuvant etoposide/cisplatin chemotherapy, in the management of primary small cell neuroendocrine carcinoma. The patient was detected no local or distant metastases, at 9 months postoperatively. However, the follow-up period of only 9 months is relatively short, which limits the long-term assessment of recurrence or survival. These findings provide a reference for clinical decision-making and underscore the potential benefits of multimodal treatment in this rare malignancy.</p>