The most common adult-onset diseases included here are autosomal dominant (AD) polycystic kidney disease (ADPKD) and AD tubulointerstitial kidney disease (ADTKD), previously known as “medullary cystic kidney disease”) due to pathogenic variants in MUC1, UMOD, REN, or HNF1B. ADTKD is characterised by microcysts rather than cysts. Tuberous sclerosis is common but results less often in kidney failure. This evidence concerns the gene HNF1B and autosomal dominant medullary cystic kidney disease with or without hyperuricemia.