Autosomal dominant tubulointerstitial kidney disease (ADTKD) is an increasingly recognized rare condition with three primary characteristics: autosomal dominant inheritance, bland urinary sediment (absence of hematuria and proteinuria) and chronic kidney disease (CKD) leading to kidney failure (need for renal replacement therapy or kidney transplantation) between 20 and 80 years of age, with a mean age of kidney failure of approximately 45 years. Pathogenic variants in UMOD, MUC1, REN, and APOA4 have been identified as causative in ADTKD families.