The increasing number or corneal and intraocular surgeries performed together with longer life expectancy, elevates the risk for developing symptomatic Fuchs' endothelial dystrophy (FED). This article reviews the current understanding of FED, and, in addition, a case of early FED is presented clinically and histopathologically. Two FED corneas from one patient were examined histopathologically using an established histological protocol for light and electron microscopy. FED is an inherited autosomal dominant corneal endothelial disorder with incomplete penetrance that is up to 3 times less likely to develop in men. Treatment options are primarily palliative while surgical intervention routinely involves a penetrating keratoplasty. In this disease process the endothelium produces excessive amounts of basement membrane material of an abnormal composition resulting in the formation of a posterior collagenous layer. Extreme accumulations of this material created mushroom-like formations, guttae, projecting into the anterior chamber. The endothelial cells were extremely thinned over the guttae, to the point where the cells may provide little more than barrier function. Despite a presumably compromised endothelial pump the corneas appeared relatively free of edema clinically and histopathologically. However, some edematous pockets deep in the basal epithelial layer were present suggesting that epithelial involvement occurs at an early stage in the disease. An early diagnosis of FED will help patients and surgeons to better elect optimal surgical timing and procedures. The current trend is to intervene surgically before the patient reaches the painful end-stage. Interestingly, in the case examined, the endothelium presumably provided only a barrier function over large areas with an apparently reduced contribution from the endothelial fluid pump and yet the corneas remained relatively clear. Future research may confirm that decompensation occurs only when complete endothelial coverage is lost. If the endothelial barrier function is more important to corneal transparency than the endothelial pump function in FED, then the relative combination of these two functions in the normal cornea should also be reassessed.