Thus, a diagnosis of autosomal dominant (AD) tubulointerstitial kidney disease-uromodulin-associated kidney disease (ADTKD-UMOD) (MIM 16,200; unknown prevalence) was made, and now, kidney transplantation from an unaffected offspring is planned for managing the patient’s condition. The gene discussed is UMOD; the disease is autosomal dominant medullary cystic kidney disease with or without hyperuricemia.