The NPHP7 gene, also known as the GLIS2 gene, contains six coding exons and encodes a Kruppel-like zinc-finger transcription factor, which has been found mutated in one consanguineous Oji-Cree Canadian family with isolated NPH in three children who developed ESRD by 8 years of age. The gene discussed is GLIS2; the disease is normal pressure hydrocephalus.