The entity of sudden cardiac death in young athletes has been known since the year 490 B.C. when young Greek soldier Phidipides had run the distance from Marathon to Athens and suddenly fell down dead. In the last twenty years, sudden death of famous athletes have attracted huge attention of medical and social community; afterwards both American and European Cardiology Societies started to publish periodically guidelines for preparticipation screening. These guidelines have focused on both identifying athletes with potential cardiovascular risk for sudden death and eligibility conditions for athletes participating in competitive sports. 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 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 idiopathic left ventricular hypertrophy. Current ACC/AHA & ESC guidelines should be widely used in order to reduce potential sudden cardiac death in young athletes.