When it was initially identified in 1945, nephronopthisis (NPHP) was known as “medullary cystic kidney disease“by Smith and Graham and “familial juvenile nephronophthisis” by Fanconi et al. The phrase “nephronophthisis” refers to the “disintegration of nephrons”. Polyuria, polydipsia, secondary enuresis, growth retardation, and anemia are among the signs of NPHP, which can appear as early as age 6. End-stage renal disease (ESRD) can begin at various ages for the uncommon infantile and teenage variants of NPHP. The prevalence of NPHP varies from 1:50,000 in Canada to almost 1 in a million in the United States, with a prevalence of 1 in 61,800 in Finland.