The sudden cardiac arrest (CA) and death of athletes are dramatic and emotionally impacting events for health professionals, family, and society. Although the practice of sport participation improves general health, physical fitness, and quality of life, intense physical exercise can be a trigger for CA and sudden death occasionally in the presence of known or unknown cardiac disorders (mainly hypertrophic cardiomyopathy) and risk factors (environment, health style, family, and genetic). The present review found that sudden death associated with CA was not such a common event in competitive athletes, but it might be an underestimated event in recreational athletes. Thus, considering the exponential increase in sport participation, both in a recreational or competitive way, and the rate of sudden CA, knowledge of implementing prevention and treatment strategies is crucial. This includes preparation of health professionals and lay people in basic life support (BLS); screening and pre-participation assessment in sport programs and health education; and promotion for the recognition of CA and early completion of BLS and rapid access to automatic external defibrillator to improve the victim survival/prognosis. Thus, the purpose of this review is to provide for health professionals and lay people the most updated information, based on current guidelines, of how to proceed in an emergency situation associated with sudden CA of young adult athletes.