Interestingly, the hemizygous contiguous gene microdeletion at 7q11.23 in Williams-Beuren syndrome (WBS; OMIM 194050) includes the CLDN3 gene (Dutra et al., 2011) and several dental manifestations have been reported in patients with WBS, including hypodontia, abnormal tooth shape (microdontia), but also hypoplastic enamel defects and higher caries susceptibility (Hertzberg et al., 1994; Axelsson et al., 2003). This evidence concerns the gene CLDN3 and Williams syndrome.