Cardiovascular diseases are the cause of death in up to 90% of athletes. The most frequent cause of death in young athletes is a sudden cardiac death (SCD). Causes of SCD among athletes are strongly correlated with age. In young athletes (< 35 years), the leading causes are congenital cardiac diseases, particularly hypertrophic cardiomyopathy and congenital coronary artery anomalies. By contrast, most of deaths in older athletes (< 35 years) are due to coronary artery disease. Although there is no possibility to decrease the risk of SCD to "zero", especially in sport competitions, clear recommendations, if available, for cardiovascular evaluation before athletic participation and side effects of sports activities, would minimize the frequency of SCD. If a specific condition of the cardiovascular system is diagnosed, it is necessary to establish the risk of SCD associated with a continual involvement in physical activities and competitive sports, and to define clearly disqualification criteria for each individual athlete.