Regular physical activity provides a variety of health benefits, including improvement in cardiopulmonary or metabolic status, reduction of the risk of coronary artery disease or stroke, prevention of cancer, and decrease in total mortality. Exercise-related cardiac events are occasionally reported during highly competitive sports activity or vigorous exercises. However, the risk of sudden death is extremely low during vigorous exercise, and habitual vigorous exercise actually decreases the risk of sudden death during exercise. The cause of sudden death is ischemic in older subjects (> or =35 years old), while cardiomyopathies or genetic ion channel diseases are important underlying pathology in younger (<35 years old) victims. The subgroup of patients who are particularly at higher risk of exercise-related sudden death may be identified in different ways, such as pre-participation history taking, physical examination and/or supplementary cardiac evaluation. Limitations exist because current diagnostic tools are not sufficient to predict a coronary artery plaque with potential risk of disruption and/or an acute thrombotic occlusion. Proper and cost-effective methods for identification of younger subjects with cardiac structural problems or genetic ion channel diseases are still controversial.