Several groups proved kidney-sparing surgery (KSS) had equivalent oncological outcomes compared with radical nephroureterectomy (RNU) for the low-risk upper urinary tract urothelial carcinoma (UTUC) patients. Whereas, the clinical efficacy of KSS for high-risk UTUC, especially for distal high-risk ureteral carcinoma, remains unclear. To evaluate the feasibility of KSS for patients with distal high-risk ureter cancer. Our study included 22 patients who diagnose the distal high-risk ureter cancer and underwent KSS between May 2012 and July 2021 in the First Affiliated Hospital of Chongqing Medical University. Overall survival (OS), confirmed as the primary endpoint of present study, was assessed by a blinded independent review committee (BIRC). The secondary endpoints included the postoperative SF-36 (the short form 36 health survey questionnaire) score, progression-free survival (PFS), postoperative complications, and so on. Overall, 17 (77.3%) and 5 (22.7%) patients underwent segmental ureterectomy (SU) and endoscopic ablation (EA), respectively. By the cut-off date, the mean OS was 76.3months (95% Cl: 51.3-101.1months) and the mean PFS was 47.0months (95% Cl: 31.1-62.8months), respectively. And the SF-36 score in a majority of patients was >300 (90.9%). This is a daring endeavor to explore the clinical efficacy of KSS in distal high-risk ureter cancer based on the high-risk UTUC criteria, which shows satisfactory results in the long-term prognosis and operation-associated outcomes. However, future randomized or prospective multicenter studies are necessary to validate our conclusions.