The combination of extreme heat, humidity and strenuous exercise is potentially challenging to the thermoregulatory system of a Thoroughbred racehorse, and under certain circumstances can cause a condition called exertional heat illness (EHI). It is difficult to predict its occurrence in an individual animal due to the combination on race day of extrinsic and intrinsic risk factors. Education regarding EHI is essential to its prevention, and lack of understanding amongst those responsible for the horse's care may in fact be the most important risk factor. The pathophysiology of EHI is similar across elite athletic species and is directly related to an elevation in core body temperature that exceeds the 'critical thermal maximum', causing widespread destruction of cells. It is not only the high core temperature but also its duration that will dictate outcome. Treatment strategies should decrease the hyperthermia to near normal levels within 30 min of onset, which will minimise adverse consequences. This review article outlines the pathophysiological changes as EHI progresses, and the case definition at each emergent level. Pharmacological treatment strategies and their rationale are presented. Aggressive cooling, however, is the key treatment and requires a technique which is both practical and effective in rapidly reducing core body temperature. The treatment paradigm is early detection, rapid assessment enabling prioritisation of individuals and aggressive cooling. Each stage is critically discussed.