The few data collections that evaluate the involvement of organ systems in horse diseases are in agreement that the locomotor, gastrointestinal and nervous systems are the sites of primary disease in the vast majority of sick horses. When compared with diseases of these organ systems, equine cardiovascular diseases occur infrequently. The most detailed and comprehensive survey of equine cardiac pathology was reported in 1972 by Else and Holmes, who summarized the gross and microscopic cardiac findings from 1500 abattoir horses. This paper reviews the pathology of the cardiovascular diseases typically encountered in horses. Most of the pathological examples are from the files of the Veterinary Medical Teaching Hospital at the University of Florida, encompassing 24 years (1978–2002) and some 6000 equine necropsy cases. Preceding the specific topics are principles of the anatomy and function of the normal equine heart. Pathological entities include equine congenital cardiovascular diseases, acquired diseases of the pericardium, myocardium, endocardium and valves, cardiac neoplasms, and common equine vascular diseases and vascular neoplasms. Extensive use is made of photographs to illustrate the features of individual case examples.