The endothelium is a monolayer of cells on the posterior corneal surface that transports water from the stroma into the anterior chamber. This movement of water counters a natural tendency for the stroma to swell and is necessary to maintain a transparent cornea. Embryologic studies, in particular the demonstration of the derivation of the endothelium from the neural crest, have provided insight into the factors that govern the response of this tissue to disease. In some species the endothelium can regenerate after injury, but in man cellular enlargement is the main mechanism of repair after cell loss. A clinical estimate of endothelial cell density and function is provided by specular microscopy, fluorophotometry and pachymetry. In this paper we review the development, structure and function of the corneal endothelium, and then consider the pathological processes that can affect this tissue.