Sudden cardiac death in athletes, although relatively rare, remains a significant clinical concern due to its occurrence in young, often asymptomatic individuals. The most common causes include inherited cardiomyopathies, coronary anomalies, arrhythmogenic right ventricular cardiomyopathy, and primary electrical disorders (channelopathies). The incidence varies across populations and sporting disciplines, reflecting both epidemiological differences and variations in data collection. Early identification of at-risk individuals is central to prevention. Pre-participation screening, particularly with the inclusion of a 12-lead electrocardiogram (ECG), significantly improves detection of underlying cardiac abnormalities compared with a history and physical examination. Risk stratification should be individualized and may incorporate clinical evaluation, imaging techniques, ambulatory monitoring, and genetic testing. Equally important is effective emergency preparedness. The implementation of structured emergency action plans, including rapid recognition of cardiac arrest, immediate cardiopulmonary resuscitation, and early use of automated external defibrillators, has been shown to markedly improve survival outcomes. Future advances in genetic testing and the development of more sophisticated AI-assisted ECG analysis may further enhance early detection and risk stratification.