Abstract: Hypertrophic cardiomyopathy(HCM) is one of the most common hereditary cardiomyopathy, the most serious outcome of which is sudden cardiac death(SCD). Early recognition and prevention may delay or avoid the onset of cardiac arrest. Therefore, it is important to identify the high-risk factors of SCD in patients with HCM and prevent cardiac arrest. At present, the recognized high risk factors mainly include family history of sudden death, non-sustained ventricular tachycardia, severe left ventricular hypertrophy, unexplained syncope, end-stage HCM with left ventricular ejection fraction < 50%, left ventricular systolic dysfunction of any size, and extensive late gadolinium enhancement. The main preventive measures involvemedical treatment, surgical septal myectomy, alcohol septal ablation, and implantable cardiac defibrillator. This article mainly focuses on the identification of high-risk factors and prevention strategies of SCD caused by HCM, and reviews the latest progress of diagnosis, treatment and prevention of HCM, to provide guidance for prevention of SCD caused by HCM.