Two genetic subtypes of Dent disease have been described to date: Dent disease 1 (OMIM #300008) is caused by pathogenic variants of Chloride voltage-gated channel 5 (CLCN5) gene which maps on chromosome Xp11.22, encoding a 746 amino-acidelectrogenic Cl−/H+ exchanger (ClC-5), and Dent disease 2 (OMIM #300555) is caused by pathogenic variants of Oculocerebrorenal syndrome of Lowe (OCRL) gene, located on chromosome Xq25, encoding inositol polyphosphate 5-phosphatase [2–4]. Here, OCRL is linked to Dent disease.