This review provides an updated overview of Fuchs' endothelial corneal dystrophy (FECD), a common bilateral corneal dystrophy that accounts for one-third of all corneal transplants in the US. FECD is caused by a combination of genetic and non-heritable factors and is characterized by 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 key features of FECD include progressive loss of corneal endothelial cells and the formation of focal excrescences (guttae) on the Descemet membrane, leading to endothelial dysfunction, decreased visual acuity, and blindness in later stages. The review summarizes the associated genes, pathophysiology, diagnosis, current therapeutic approaches, and future treatment perspectives. Historically, penetrating keratoplasty has been the gold standard for treatment, but new surgical techniques have improved visual recovery and reduced complications. Genome editing using CRISPR-Cas9 is being explored as a potential future therapeutic solution. FECD has a higher prevalence among Caucasians, Icelandic and Singaporean Chinese populations, and is more common in women, especially in the late-onset variant. The disease is associated with other conditions such as keratoconus, axial hyperopia, glaucoma, macular drusen, age-related macular degeneration, and myotonic dystrophy. The cornea, composed of five layers, functions as the first defense barrier of the eye. The corneal endothelium (CE) maintains the cornea's hydration and prevents edema. In FECD, the loss of cell density and abnormal changes in endothelial cells lead to reduced corneal dehydration and vision impairment. The review highlights the importance of understanding the pathophysiology and ongoing research into regenerative therapies for corneal endothelium to improve the management and care of FECD.This review provides an updated overview of Fuchs' endothelial corneal dystrophy (FECD), a common bilateral corneal dystrophy that accounts for one-third of all corneal transplants in the US. FECD is caused by a combination of genetic and non-heritable factors and is characterized by 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 key features of FECD include progressive loss of corneal endothelial cells and the formation of focal excrescences (guttae) on the Descemet membrane, leading to endothelial dysfunction, decreased visual acuity, and blindness in later stages. The review summarizes the associated genes, pathophysiology, diagnosis, current therapeutic approaches, and future treatment perspectives. Historically, penetrating keratoplasty has been the gold standard for treatment, but new surgical techniques have improved visual recovery and reduced complications. Genome editing using CRISPR-Cas9 is being explored as a potential future therapeutic solution. FECD has a higher prevalence among Caucasians, Icelandic and Singaporean Chinese populations, and is more common in women, especially in the late-onset variant. The disease is associated with other conditions such as keratoconus, axial hyperopia, glaucoma, macular drusen, age-related macular degeneration, and myotonic dystrophy. The cornea, composed of five layers, functions as the first defense barrier of the eye. The corneal endothelium (CE) maintains the cornea's hydration and prevents edema. In FECD, the loss of cell density and abnormal changes in endothelial cells lead to reduced corneal dehydration and vision impairment. The review highlights the importance of understanding the pathophysiology and ongoing research into regenerative therapies for corneal endothelium to improve the management and care of FECD.