Recent studies have reported ECG anomalies and a high prevalence of exercise-related arrhythmias among well trained, apparently healthy endurance athletes with superior levels of cardiorespiratory fitness. The occurrence of sudden and premature cardiac deaths in amateur and professional athletes, who appear to embody all of the virtues of health and fitness, ahs raised our consciousness regarding the underlying atherosclerotic or nonatherosclerotic causes, and the need for, and extent of, preparticipation screening in competitive athletes. It appears that strenuous physical activity may trigger acute cardiovascular events in some athletes. Coronary artery disease is the most frequent autopsy finding in those over the age of 35 years who die suddenly. In contrast, structural cardiovascular abnormalities, including hypertrophic cardiomyopathy and malformations of the coronary arteries, are the major cause of sudden death in younger athletes. This article reviews these issues, with specific reference to the assessment of cardiorespiratory fitness, legal and prohibited performance-altering medications, the pathophysiological basis of exertion-related untoward events, the athlete at risk, limitations of conventional screening programmes and contemporary recommendations to identify latent cardiovascular disease in athletic populations.