The sudden death in athletes is, in the vast majority of cases, related to ventricular fibrillation, often in a subject with unknown cardiovascular abnormality; this dramatic event has a significant impact on society and the medical profession. We conducted through a literature review an analysis of data on sudden cardiac death of rhythmic origin in athletes; sudden death may be cardiovascular in 95.3% of cases and related to ventricular arrhythmia in 88% cases. The main causes are: hypertrophic cardiomyopathy, congenital anomalies of coronary arteries, and arhythmogenic right ventricular dysplasia for athletes under 35 years, and atherosclerosis beyond 35 years. Prevention is based on three main areas: the medical assessment and screening for cardiovascular disease; the chain of survival; the education of the athlete and the public. All these measures should improve significantly the survival prognosis of patients suffering from these accidents.