Nowadays, sports are a wonderful mean for social success, and the high-level athlete is the symbol of a perfect hygiene of life. Despite this, the occurrence of unexplained sudden death (SD) is not exceptional, especially during training and competition. In this context, it is important to intensify medical controls for these athletes, especially in a very early phase, in order to detect subjects at risk. In case of detection of a cardiac disease prone to cardiovascular or arrhythmic event, the practice of any high-level sportive activity or even any sustained sportive activity must be forbidden without hesitation, with the aim of protecting these subjects. Even though a total interdiction of sports practice can be a tough decision to be accepted, it should prevail on the dramatic consequences of sudden death. Physicians' responsibility issues in the screening and management of competition or leisure-time sportsmen are of high importance since in case of sudden death, the physician and the medical community liabilities can be considered. As a consequence, the medical community set up recommendations on the screening, treatment and even interdiction of sportive activity for athletes, which should also be applied to leisure-time sportsmen. In the first part of this article, the different causes (especially the arrhythmia-related) of sudden death occurring in sportsmen are reviewed. In the second part, the recommendations on practice of high-level sports in case of arrhythmia or genetic arrhythmic cardiac disease are summarized.