BackgroundThe purpose of this study was to conduct a population‐based study to determine the prognosis of renal cell carcinoma (RCC) in children and adolescents.MethodsPatients with RCC who were registered in the Surveillance, Epidemiology, and End Results (SEER) program between 2000 and 2018 had their demographic and clinical characteristics evaluated retrospectively. The log‐rank test was used to compare survival curves. Kaplan–Meier estimates were used to generate survival curves based on various factors. To identify factors associated with overall survival, Cox proportional‐hazards regression was used.ResultsA total of 251 patients were enrolled in the study. For all patients, the overall survival (OS) rates at 3‐ and 5‐ year were 93.5% and 92.0%, respectively. A multivariable study revealed that the following factors were independently associated with overall survival: sex, race, histologic type, SEER stage, AJCC stage, and type of surgery. Cox analysis showed that white patients had the lowest risk of mortality (hazard ratio (HR) 2.58, 95% confidence interval (CI), 1.33–4.99; P = 0.005), compared with black patients. Patients having metastatic disease had significantly higher mortality risk (HR 43, 95% CI, 14.8–125; P < 0.001) than the patients with localized tumour.ConclusionsOur study emphasizes the importance of race, SEER stage, and surgery in the prognosis of paediatric RCC, providing valuable epidemiological evidence for clinical practice. Economic studies assessing a race/ethnic group specific strategy are also required.