Although regular aerobic physical activity increases exercise capacity and plays a role in both primary and secondary prevention of a variety of chronic disorders, competitive physical exercise is associated with a significant increase of risk of sudden death in athletes, especially adolescents and young adults. Several pathogenetic mechanisms have been speculated, including silent cardiovascular conditions, mostly cardiomyopathy, premature coronary artery disease and congenital coronary anomalies. Uneventful events, especially commotio cordis, and abuse of unfair and dangerous performance-enhancing drugs, are also claimed as potential causes. Although identification of athletes at major risk and prevention of adverse events seems the more pervasive strategy, guidelines for screening athletes differ widely on international basis and even among the different Sport federations. The aim of this review was to compile the current knowledge on the prevalence and the most common causes of sudden death in sportsmen, providing an overview of the guidelines for pre-participation screening.