Abstract Background Health-related quality of life is a very important issue in children with end-stage renal disease and their family. Moreover, this can be a lifelong problem. In this study, we performed a cross-sectional investigation of the health-related quality of life in Korean children, undergoing renal replacement therapies, such as dialysis and renal transplantation. Findings We validated the Korean version of the PedsQL 3.0 End-Stage Renal Disease Module by comparing with the PedsQL 4.0 Generic Core Scales. A total of 92 pediatric patients with end-stage renal disease, aged 2–18 year old, were enrolled in four teaching hospitals in Korea. The module was acceptable for both parent proxy-report and child self-report. The response rate was acceptable, since no reminders were delivered. A large proportion of the responders answered >90% of the items, which suggests a good face validity. The PedsQL 4.0 Generic Core Scales and the PedsQL 3.0 End-Stage Renal Disease Module showed minimal missing values in the current study, which supported feasibility. The validation analyses revealed acceptable floor and ceiling effects and an acceptable construct validity. Conclusions The PedsQL 3.0 End-stage Renal Disease Module may be useful as an end-stage renal disease -specific instrument in the evaluation of the health-related quality of life in Korean children; however, a larger, longitudinal prospective study is needed.