Fuchs' endothelial dystrophy of the cornea is a significant cause of corneal blindness in the United States. The disease is characterized by a slow, continuous loss of morphologically and physiologically altered endothelial cells, eventually leading to corneal edema. The endothelial cells synthesize a thickened Descemet's membrane with focal excrescences of altered basement membrane material (guttae). This review details the epidemiological, clinical, and laboratory data that have accumulated on Fuchs' dystrophy. Several hypotheses regarding the pathogenesis of Fuchs' dystrophy are discussed, including the possible influences of aberrant embryogenesis, hormones, and injury on the development of the disease. The current state of medical and surgical management is summarized, along with the future prospects for treatment.