Dent disease is an X-linked hereditary tubular disorder, the causative genes of which are CLCN5 and OCRL. Approximately 60% of clinically diagnosed cases of Dent disease are Dent disease-1 and around 15% are Dent disease-2, while in the remaining 20–25%, no genetic abnormalities are identified [37]. The gene discussed is CLCN5; the disease is Dent disease.