Hypertrophic cardiomyopathy (HCM) is a hereditary cardiomyopathy. Most HCM patients develop mild symptoms throughout their lives. However, they also are at risk of ventricular arrhythmia and sudden cardiac death (SCD), with a prevalence of 80/100000 in Chinese adults, and SCD is the most serious complication. Identifying the risk of SCD in HCM patients can improve their prognosis outcome. In this article, based on the current guidelines and recent studies on HCM and SCD, we review the pathological mechanism, identification of risk factors and prevention of SCD in HCM patients.