Sudden death during sports activities is extremely rare in athletes and sportsmen. Its occurrence was calculated at 0.77 to 13 deaths per 100,000 sportsmen/year. The most frequent causes were coronary heart disease, coronary muscular bridges, congenital coronary artery anomalies, subarachnoid hemorrhage, hypertrophic cardiomyopathy, arrhythmogenic right ventricular dysplasia, Marfan syndrome, aortic coarctation, myocarditis, pulmonary embolism, aortic stenosis, mitral valve prolapse and WPW syndrome. Clinical examination nearly identifies all cases of aortic stenosis, mitral valve prolapse with regurgitation, and aortic coarctation but misses the majority of cases of hypertrophic cardiomyopathy and coronary artery diseases. The use of Chest x-rays, ECG, Stress Test and Echocardiogram will provide the identification of most cases with increased risk of death. Although costs are not limited for professional athletes, this strategy does not totally overcome the problem because diagnostic errors are frequent (false positives and false negatives). Therefore it is important to admit the failure of these screening procedures and the necessity to adapt the strategy to cost-efficiency and time-efficiency in this population.