The sudden and unexpected death in young athletes is always a tragic event. At the age < 35 years distinct structural cardiac disorders such as hypertrophic cardiomyopathy, inflammatory disorders, coronary artery anomalies, as well as conditions without structural cardiac abnormalities such as primary electrical diseases (Brugada's syndrome, long QT, short QT syndrome and catcholaminergic polymorphic ventricular tachycardia) are important causes of sudden death. At the age > 35 years coronary artery disease is the most common cause of cardiac death in athletes. In this review we give an overview about actual evaluation of competitive athletes and prevention of sudden cardiac death.