AbstractIntroductionHealth‐related quality of life (HRQoL) studies demonstrate the impact of end‐stage renal disease (ESRD) on the physical and psychosocial development of children. While several instruments are used to measure HRQoL, few have standardized domains specific to pediatric ESRD. This review examines current evidence on self and proxy‐reported HRQoL among pediatric patients with ESRD, based on the Pediatric Quality of Life Inventory (PedsQL) questionnaires.MethodsFollowing PRISMA guidelines, we conducted a systematic review and meta‐analysis on HRQoL using the PedsQL 4.0 Generic Core Scale (GCS) and the PedsQL 3.0 ESRD Module among 5‐ to 18‐year‐old patients. We queried PubMed, Embase, Web of Science, CINAHL, and Cochrane databases. Retrospective, case‐controlled, and cross‐sectional studies using PedsQL were included.FindingsOf 435 identified studies, 14 met inclusion criteria administered in several countries. Meta‐analysis demonstrated a significantly higher total HRQoL for healthy patients over those with ESRD (SMD:1.44 [95% CI: 0.78–2.09]) across all dimensional scores. In addition, kidney transplant patients reported a significantly higher HRQoL than those on dialysis (PedsQL GCS, SMD: 0.33 [95% CI: 0.14–0.53]) and (PedsQL ESRD, SMD: 0.65 [95% CI: 0.39–0.90]) concordant with parent‐proxy reports.DiscussionPatients with ESRD reported lower HRQoL in physical and psychosocial domains compared with healthy controls, while transplant and peritoneal dialysis patients reported better HRQoL than those on hemodialysis. This analysis demonstrates the need to identify dimensions of impaired functioning and produce congruent clinical interventions. Further research on the impact of individual comorbidities in HRQoL is necessary for developing comprehensive, integrated, and holistic treatment programs.