Young competitive athletes are perceived by the general population to be the healthiest members of society. The possibility that highly trained high school and college athletes may have a potentially serious cardiac condition that can predispose to life-threatening dysrhythmias or sudden cardiac death (SCD) seems paradoxical. 1 The occurrence of SCD in young athletes from dysrhythmias is an uncommon but highly visible event. Media reports of sudden death in athletes have intensified the public and medical interest in medical, ethical, and legal issues related to cardiac disorders in the athlete. Developing screening strategies to identify conditions associated with sudden death has been the focus of attention of experts in the fields of arrhythmology and sports medicine and has resulted in Consensus Statements and Guidelines for evaluation of athletes. These guidelines provide information and recommendations for detection, evaluation, and management of athletes with cardiovascular disorders and criteria for eligibility and disqualification from participation in high-intensity and competitive sports. Differentiating normal exercise-induced physiologic changes in the heart from pathological conditions associated with sudden death is critical for developing screening strategies to identify athletes at high risk. This article discusses a case report of sudden cardiac death in an athlete followed by a brief review of various causes of cardiac dysrhythmias in young athletes and recommendations for screening and management of athletes with cardiovascular diseases.