During the last 20 years, the principal objective in hypertrophic cardiomyopathy research has been the refinement of algorithms for the identification and treatment of patients at risk of sudden death. Sudden death is an important problem in hypertrophic cardiomyopathy, with an incidence of 4-6% in referral populations and approximately 1% in non-referral centers and because it affects young and often asymptomatic patients. We now know that hypertrophic cardiomyopathy is not a single disease, but a group of diseases caused by mutations in genes encoding different sarcomeric proteins. The phenotypic expression depends on multiple modifying genetic and environmental factors. Even though genetic testing is not presently a practical approach in hypertrophic cardiomyopathy risk stratification, it is important to consider new genetic data in the prognostic evaluation of patients. In this paper, we review the published data on risk stratification in hypertrophic cardiomyopathy and we set forth our opinion with regard to the available therapeutic options and their indications in the prevention of sudden death.