Malignant renal tumours represent 5% of childhood cancers and include types with likely different aetiology: Wilms tumour (WT), rhabdoid renal tumour, kidney sarcomas and renal carcinomas. WT is the most common renal tumour in children, previously shown to vary internationally and with ethnicity. Using the comprehensive database of the International Incidence of Childhood Cancer study (IICC), we analysed global variations and time trends in incidence of renal tumour types in children (age 0‐14 years) and adolescents (age 15‐19 years). The results were presented by 14 world regions, and five ethnic groups in the US. We included 15 320 renal tumours in children and 800 in adolescents reported to the 163 contributing registries during 2001‐2010. In children, age‐standardised incidence rate (ASR) of renal tumours was 8.3 per million (95% confidence interval, CI = 8.1, 8.4); it was the highest in North America and Europe (9‐10 per million) and the lowest in most Asian regions (4‐5 per million). In the US, Blacks had the highest ASR (10.9 per million, 95% CI = 10.2, 11.6) and Asian and Pacific Islanders the lowest (4.4 per million, 95% CI = 3.6, 5.1). In adolescents, age‐specific incidence rate of renal tumours was 1.4 per million (95% CI = 1.3, 1.5). WT accounted for over 90% of all renal tumours in each age from 1 to 7 years and the proportion of renal carcinomas increased gradually with age. From 1996 to 2010, incidence remained mostly stable for WT (average annual percent change, AAPC = 0.1) and increased for renal carcinomas in children (AAPC = 3.7) and adolescents (AAPC = 3.2). Our findings warrant further monitoring. What's new? Based on more than 16,000 incident cases in the period 2001‐2010, this study offers the most complete overview to date of the worldwide patterns of renal tumours in children and adolescents. Using the comprehensive International Incidence of Childhood Cancer database, the authors also describe the distribution of rare entities such as rhabdoid renal tumour or kidney sarcomas. The results indicate the stable incidence of Wilms tumour, the most common renal tumour in children, consistently with a likely genetic origin. The rising incidence of renal carcinomas with age and over time is likely caused by environmental risk factors, warranting further monitoring.