<b>Objectives and Study:</b> Congenital chloride diarrhea (CCD) is a rare, autosomal recessive disorder caused by mutations in the <i>SLC26A3</i> gene encoding a transmembrane chloride/bicarbonate ion exchanger mainly expressed in the apical brush border of the ileal and colonic epithelium. The gene discussed is SLC26A3; the disease is Congenital chloride diarrhea.