Congenital hereditary endothelial dystrophy (CHED) [1] is a rare autosomal recessive corneal endothelial dystrophy that presents as early onset childhood corneal clouding, typically manifesting at birth or immediately thereafter. It has a high predilection in areas with high rates of parental consanguinity and is commonly seen in Asian and Middle Eastern countries. CHED is characterized by bilateral, diffuse, noninflammatory, and ground glass cloudy cornea, and a mutation of SLC4A11 [2], an abundant corneal solute transporter, has been attributed to its causation. SLC4A11 is a large multi-domain integral membrane protein encoded by the SLC4A11 gene, which was earlier thought to be a bicarbonate transporter-related protein 1 (BTR1) [3] but was later found to be a sodium-borate cotransporter [4]. CHED cases have also been associated with progressive, postlingual sensorineural hearing loss (SNHL), a condition known as the Harboyan Syndrome [23]. The ocular manifestation is indistinguishable from the normal forms of CHED, and the overlap is due to the presence of SLC4A11 gene products in the spiral ligament fibrocytes of the inner ear.