ABSTRACT Introduction and importance: Upper tract urothelial carcinoma (UTUC), including renal pelvis cancer and ureteral cancer, is highly prevalent in people aged 70 ˜ 90 years. Immune checkpoint inhibitor therapy is a major breakthrough in the field of oncology and is an emerging anti-tumor treatment modality after chemotherapy, targeted therapy and anti-angiogenic therapy. Here we presented a UTUC case who benefited from treatment of immunotherapy combined with chemotherapy in neoadjuvant therapy. Case presentation: A 66-year-old male patient presented on July 2, 2021, with a 3-month history of left-sided abdominal pain and a 2-month history of gross hematuria. To confirm the diagnosis, the treatment of left ureteroscopy was performed under general anesthesia on July 7, 2021. Biopsy pathology revealed urothelial carcinoma, clinical stage cT3-4N1M0, high-risk. Four cycles of neoadjuvant therapy were preoperatively administered with immunotherapy combined with chemotherapy every 21 days. Partial response (PR) was confirmed via imaging after the 4th cycle. The patient underwent radical surgery on November 11, 2021. Postoperative diagnosis: left renal pelvis cancer, pT3N0M0, high risk. Adjuvant therapy was continued after surgery with 4 cycles of GC chemotherapy. After chemotherapy, CTU reexamination and cystoscopy was performed and show no signs of the carcinoma returning. Discussion: Immune checkpoint inhibitors have become first-line therapy for patients with metastatic UC who are not candidates for cisplatin chemotherapy. However, the current literature is inconclusive on the efficacy of neoadjuvant therapy with preoperative immunotherapy combined with chemotherapy. We discuss clinical outcomes and literature reviews for these patients. Conclusion: It’s feasible and safe in PD-L1-positive locally advanced UTUC patients that preoperative neoadjuvant PD-1 inhibitor immunotherapy combined with GC chemotherapy was selected for 4 cycles, after neoadjuvant therapy, further radical surgery was performed, and adjuvant chemotherapy was performed after surgery. Highlight