Fuchs' endothelial corneal dystrophy (FECD) is the most common bilateral corneal dystrophy, accounting for one-third of all corneal transplants in the US. It is caused by a combination of genetic and non-heritable factors and has two types: early-onset FECD, which affects individuals from an early age and is more severe, and late-onset FECD, which is more common and typically manifests around the age of 40. The hallmark findings of FECD include progressive loss of corneal endothelial cells and the formation of focal excrescences (guttae) on the Descemet membrane. These pathophysiological changes result in progressive endothelial dysfunction, leading to decreased visual acuity and blindness in later stages. FECD is an autosomal dominant hereditary disease with a worldwide prevalence that varies among different ethnic groups, with higher frequency observed among Caucasians, inhabitants of Iceland, and Singaporean-Chinese populations. It is more prevalent in women, with a 2–3:1 ratio to males, especially for the late-onset variant. FECD has a significant correlation with age, with individuals over 40 being the most susceptible. It is also associated with various diseases, including keratoconus, axial hyperopia, glaucoma, macular drusen, age-related macular degeneration, and myotonic dystrophy. The cornea is composed of five layers, with the corneal endothelium (CE) playing a crucial role in maintaining corneal dehydration. The CE is a single layer of hexagonal cells that maintain the cornea dehydrated through the Na+/K+ ATPase pump and aquaporin 1 (AQP-1) channels. In FECD, the loss of cell density leads to endothelial cell migration and abnormal changes in morphology, resulting in reduced ability to dehydrate the cornea. Current treatment options include penetrating keratoplasty, but new surgical techniques have improved visual recovery and reduced complications. Genome editing using CRISPR-Cas9 is being studied as a potential future therapeutic solution for FECD. With the characterization of FECD-related genes and ongoing research into regenerative therapies, there is hope for significant improvements in the management and care of the disease.Fuchs' endothelial corneal dystrophy (FECD) is the most common bilateral corneal dystrophy, accounting for one-third of all corneal transplants in the US. It is caused by a combination of genetic and non-heritable factors and has two types: early-onset FECD, which affects individuals from an early age and is more severe, and late-onset FECD, which is more common and typically manifests around the age of 40. The hallmark findings of FECD include progressive loss of corneal endothelial cells and the formation of focal excrescences (guttae) on the Descemet membrane. These pathophysiological changes result in progressive endothelial dysfunction, leading to decreased visual acuity and blindness in later stages. FECD is an autosomal dominant hereditary disease with a worldwide prevalence that varies among different ethnic groups, with higher frequency observed among Caucasians, inhabitants of Iceland, and Singaporean-Chinese populations. It is more prevalent in women, with a 2–3:1 ratio to males, especially for the late-onset variant. FECD has a significant correlation with age, with individuals over 40 being the most susceptible. It is also associated with various diseases, including keratoconus, axial hyperopia, glaucoma, macular drusen, age-related macular degeneration, and myotonic dystrophy. The cornea is composed of five layers, with the corneal endothelium (CE) playing a crucial role in maintaining corneal dehydration. The CE is a single layer of hexagonal cells that maintain the cornea dehydrated through the Na+/K+ ATPase pump and aquaporin 1 (AQP-1) channels. In FECD, the loss of cell density leads to endothelial cell migration and abnormal changes in morphology, resulting in reduced ability to dehydrate the cornea. Current treatment options include penetrating keratoplasty, but new surgical techniques have improved visual recovery and reduced complications. Genome editing using CRISPR-Cas9 is being studied as a potential future therapeutic solution for FECD. With the characterization of FECD-related genes and ongoing research into regenerative therapies, there is hope for significant improvements in the management and care of the disease.