Purpose: This study aimed to determine the predictive value of concomitant carcinoma in situ (CIS) for cancer-specific survival (CSS) in patients with upper-tract urothelial carcinoma (UTUC) undergoing radical nephroureterectomy. Methods: This was a retrospective analysis of the clinicopathological features in a cohort of 126 patients with localized lymph node–negative UTUC who underwent radical nephroureterectomy with bladder-cuff excision in a tertiary medical center between January 1, 2007, and June 30, 2018. Cox proportional hazards models were used to identify risk factors for CSS in UTUC. Additionally, Kaplan–Meier analyses were performed in subgroups according to pathological tumor stage. Results: The median age and follow-up duration were 70 years and 3.5 years, respectively. Concomitant CIS, which was present in 21% of the patient specimens, was not associated with most clinical or pathological features, except for lymphovascular invasion and multifocality. Concomitant CIS (adjusted hazard ratio (HR)=4.64, 95% confidence interval (CI) 1.78–12.06, p=0.002) and pathological tumor stage (adjusted HR=4.07, 95% CI 1.99–8.31, p<0.001) were significantly associated with CSS in the multivariate Cox regression model. In the subgroup analysis, concomitant CIS in patients with locally advanced (pT3/pT4) UTUC was associated with significantly worse CSS compared to those without CIS (HR=3.83, 95% CI 1.20–12.21). Conclusion: The pathological presence of concomitant CIS was independently associated with poor CSS in patients with localized lymph node–negative UTUC undergoing radical nephroureterectomy. These findings provide crucial information relevant for postoperative patient counseling, use of adjuvant therapy, follow-up intensity, and clinical trial enrollment. Level of evidence Level II.