Knowledge of sudden cardiac death in young athletes is imperative for all physicians and allied health professionals. The complete differential diagnosis of a young patient with sudden cardiac arrest will result in proper work-up and treatment. In this article, we review several etiologies of sudden cardiac death, including hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, Wolff-Parkinson-White syndrome, long QT syndrome, Brugada syndrome, and commotio cordis. Clinical findings, work-up, treatment, long-term management, and athlete preparticipation screening guidelines are discussed.