We report a case of invasive small-cell carcinoma (SCC) of the ureter successfully treated by neoadjuvant chemotherapy and laparoscopic nephroureterectomy. SCC of the ureter is an extremely rare condition characterized by aggressive behaviour. A 70-year-old male presented with left flank pain; he was diagnosed with SCC of the ureter, cT3N0M0, by ureteroscopic biopsy. The patient received 3 cycles of neoadjuvant chemotherapy with cisplatin and irinotecan (IP) and underwent laparoscopic nephroureterectomy. The pathological diagnosis was urothelial carcinoma, high grade, without a small-cell component. The pathological stage was down-staged to pT2N0M0. Adjuvant chemotherapy was not performed. The patient has been free of local recurrence or distant metastasis for 38 months postoperatively. This is the first reported case of primary invasive SCC of the upper urinary tract treated by neoadjuvant chemotherapy followed by nephroureterectomy.