Sudden cardiac death (SCD) is a rare but devastating event among young people. The precise scale of the event remains undefined. This uncommon but catastrophic event usually is proved to be the consequence of varied unsuspected congenital or acquired cardiovascular diseases. Hypertrophic cardiomyopathy and coronary artery anomalies are the two most frequent causes of SCD. Most commonly, SCD resulting from these diseases occurs during or immediately after exercise. This report highlights the prevalence and spectrum of SCD-predisposing diseases and how they are affected by athletic participation. In addition, disease-specific guidelines for sports participation are addressed.