The DNA methylation urine test, a non-invasive early detection method for upper tract urothelial carcinoma (UTUC), is currently in full swing. This study aimed to systematically assess its diagnostic performance on UTUC.PubMed, Scopus, Embase, and Cochrane were our main databases when searching articles published from January 2000 to December 2023. Sensitivity and specificity were study primary endpoints. I2 was used to evaluated heterogeneity, meanwhile subgroup and meta-regression analyses were adopted to investigated the source of heterogeneity. Sensitivity analysis was performed to evaluate the result robustness, while Deeks' funnel plot asymmetry test was for the publication bias.Nine studies with 1326 patients were included. The pooled sensitivity was 0.89 (95% confidence interval (CI), 0.83-0.93) and specificity were 0.91 (95% CI, 0.83-0.96). The area under the receiver operating characteristic curve was 0.96 (95% CI, 0.93-0.97). Substantial heterogeneity was found during the data synthesis, whereas the pooled results remained robust in the sensitivity analysis. None of the potential covariates-urine sample collection method, population, country, methylation test method, or tumor grade-could account for the heterogeneity.DNA methylation urine test is a promising method with high efficiency for UTUC early detection. Nevertheless, owing to the significant heterogeneity, more well-organized studies are warranted to further explore its diagnostic efficiency and application context.Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc.