Endothelial-to-mesenchymal transition (EndMT) is a physiological process that is equally important during development and under certain pathological conditions in adult tissues. The last decade has witnessed a remarkable explosion of information about EndMT from molecular mechanisms responsible for its development to its role in various disease processes. The emerging picture is that of a complex set of interactions that underly pathophysiological basis of some of the most deadly and intractable diseases. This mini review brings together recent advances and attempts to present a unified view of this complex field.