The rising global burden of chronic renal disease, the high cost of providing renal replacement therapies, and renal disease also being a risk factor for cardiovascular disease is increasing focus on renal disease prevention. This article focuses on the aspects of renal disease (specifically poststreptococcal glomerulonephritis [PSGN] and chronic kidney disease [CKD]) in Indigenous populations in Australia, New Zealand, Canada, and the United States that diverge from those typically seen in the general population of those countries. The spectrum of renal and many other diseases seen in Indigenous people in developed countries is similar to that seen in developing countries. Diseases like PSGN that have largely disappeared in developed countries still occur frequently in Indigenous people. CKD during the childhood years is due to congenital anomalies of the kidney and urinary tract in up to 70% of cases and occurs later in polycystic kidney disease and childhood-onset diabetes. Several risk factors for CKD in adulthood are already present in childhood.