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. The gene discussed is UMOD; the disease is autosomal dominant medullary cystic kidney disease with or without hyperuricemia.