Here, we report the identification of sex differences in a cohort of 177 individuals with Corneal Hereditary Endothelial Dystrophy (CHED), a rare corneal endothelial dystrophy associated with biallelic SLC4A11 gene mutations, and in a Slc4a11<sup>-/-</sup> mouse model of CHED.<h4>Methods</h4>Central corneal thickness (CCT) was measured in individuals with CHED and in Slc4a11<sup>-/-</sup> and Slc4a11<sup>+/+</sup> mice to identify a correlation between sex and the degree of corneal edema. This evidence concerns the gene SLC4A11 and congenital hereditary endothelial dystrophy of cornea.