Sudden death in young sportspeople is a rare but important event. In the last twenty years, sudden deaths of athletes have attracted huge attention of the medical and social community. Such events assume a high public profile because of the generally held perception that trained athletes constitute the healthiest segment of our society, and such events do not seem logical. Structural and functional abnormalities causing sudden cardiac death in young athletes have been identified by autopsy-based studies. Unrecognized congenital cardiovascular abnormalities associated with excessive physical effort create a background for electrophysiological instability and occurrence of malignant ventricular tachyarrhythmia and consequent death. The most frequent causes of sudden cardiac death in young athletes include hypertrophic cardiomyopathy, anomalies of the coronary arteries, and arrhythmogenic right ventricular dysplasia. Sudden deaths on the athletic field occur most commonly in high school athletes. Although they are believed to be uncommon, the frequency with which such deaths occur remains largely unknown. A proportion of these deaths are potentially preventable, which has fuelled an ongoing scientific debate on the merits and timeliness of preparticipation screening for competitive athletes. Preparticipation screening has already been implemented on different levels in many countries. This review was made using materials obtained by searching relevant literature on the topic, using the keywords “sudden death”, “cardiac sudden death”, “young athletes”, “mass screening”, and “hypertrophic cardiomyopathy”. Articles published in the last fifteen years were mainly used.