Abstract: 　Chloride ion is an important anion in organisms, managing various physiological events. A particular gene mutation leads to involved channel deficiency and to develop channelopathy. In kidney, different chloride channels distribute along certain fractions of the renal tubule, located at apical and basolateral membranes of tubular epithelial cells. Previous studies discovered that voltage-sensitive chloride channels in kidney are associated with Bartter syndrome and Dent’s disease. In addition, the kidney can be involved by cystic fibrosis resulting from dysfunction of cystic fibrosis transmembrane conductance regulator. In this review, the function and mechanism of chloride channels in maintenance of normal renal function, and the renal diseases caused by related gene defects were discussed.