To describe the trends in end-stage kidney disease (ESKD) in children in Australia and New Zealand over time and across different ages, we analyzed data from the Australia and New Zealand Dialysis and Transplant Registry (ANZDATA). A total of 1,485 children aged less than 18 years received renal replacement therapy (RRT) during the period from 1963 to 2006, of which children 55.6% were male. The incidence of ESKD increased over the first two decades but has been stable at 8 per million since the mid-1980s. The prevalence of ESKD continues to increase in all age groups, especially among older children, and is currently 50 per million in those aged less than 18 years. The cause of ESKD over the entire cohort was one-third each for glomerulonephritis (32.5%), structural anomalies (hypoplasia/dysplasia, posterior urethral valves or reflux nephropathy, 35.8%), and cystic disease or other conditions (31.7%). Proportionately, glomerulonephritis is becoming less common. Overall, 50% of children were commenced on peritoneal dialysis as the initial RRT modality, 30% were started on hemodialysis, and 20% underwent transplantation pre-emptively. The proportion of children receiving transplants has not increased over time.