It is caused by pathogenic variants in at least 5 different genes: uromodulin (UMOD), mucin 1 (MUC1), hepatocyte nuclear factor 1 beta (HNF1B), renin (REN), and the alpha subunit of the endoplasmic reticular membrane translocon (SEC61A1) and have been categorized as ADTKD-UMOD, ADTKD-MUC1, ADTKD–REN, ADTKD-HNF1B, ADTKD-SEC61A1 or ADTKD-NOS (not otherwise specified), respectively [1–5]. The gene discussed is HNF1B; the disease is autosomal dominant medullary cystic kidney disease with or without hyperuricemia.