Tubular dysgenesis in patients with deleterious variants in HNF1B is appreciated by the clinical term “HNF1B associated Autosomal dominant Tubular Kidney Disease” (HNF1B-ADTKD), which is characterized by chronic kidney disease including hyperuricemia with or without gout, hypokalemia, hypomagnesemia, and polyuria [12]. The gene discussed is HNF1B; the disease is autosomal dominant medullary cystic kidney disease with or without hyperuricemia.