Hypertrophic cardiomyopathy is an important cause of sudden death in young and asymptomatic patients. Young athletes and patients with unexplained sudden death, including their relatives, should be screened for its presence. Risk stratification identifies a high-risk group, in which preventive measures should be taken to reduce the risk for sudden death: moderate to heavy physical activity has to be avoided and arrhythmias with haemodynamic impact should be rigorously prevented.