Sudden cardiac arrest (SCA) and sudden cardiac death (SCD) during exercise are rare but devastating for a patient and family. The objectives of this review are to provide both school and sport-events' personnel with an overview of the types of preventions, such as screening tests and cardiac emergency response plans, describe the recommended screening tests and cardiac emergency response plans during exercise, and describe current concerns and challenges in the prevention of SCA or SCD. A literature review was performed. No intervention study was identified. Thus, based on findings from observational studies, the types of prevention against SCA and SCD have been reviewed, and the recommendations for prevention were discussed. The 12-Element AHA Recommendations for Preparticipation Cardiovascular Screening of Competitive Athletes was strongly recommended as a screening test. The 12-lead ECG remained controversial for use as a preparticipation program. Genetic testing was not appropriate for prevention. In addition, concerns and challenges for future studies were discussed. An observational study with rigorous methodology and analysis is feasible and could provide a good understanding as a controlled trial.