Congenital endothelial dystrophy (CHED) manifests either in infancy or in young childhood with a cloudy cornea, light sensitivity, tearing, and sometimes nystagmus. Two types of CHED are recognized CHED 1 (autosomal dominant) and CHED 2 (autosomal recessive). CHED 1 is a rare subtype, characterized by swelling (edema) of the cornea, pain, and corneas that are clear at birth, but become cloudy during early infancy. Patients with CHED1 usually require a penetrating keratoplasty, it carries a good visual prognosis, even when done late in life.