Regular physical activity provides significant health benefits and reduces the risk of premature death from any cause, including cardiovascular diseases (CVD). However, physical activity may provoke sudden cardiac death (SCD), especially in presence of unrecognized diseases. It is essential to identify risk factors that contribute to SCD in athletes and to implement effective prevention of such episodes. For this purpose, SCD registries are being created, medical terminology is being unified, and schedules of medical examination are being developed. The best strategy to combat SCD during sport activities is a combination of primary prevention by screening examination to identify athletes with CVD at risk and a proper planning of first aid measures during sports events, including the availability of an automatic external defibrillator on site.