Hypertrophic cardiomyopathy (HCM) is a genetically determined, primary myocardial disease associated with an increased risk for sudden cardiac death during physical exertion. In the United States, HCM is the most frequent cause of exertion-related sudden cardiac death (SCD). Current recommendations provided by the 26th Bethesda Conference entitled Recommendations for Determining Eligibility for Competition in Athletes with Cardiovascular Abnormalities restrict participation for patients with HCM to sports requiring low levels of dynamic and isometric exertion. Such recommendations are prudent given the association of the disease with disastrous cardiovascular consequences. Nevertheless, because the prognosis of HCM and its risk of sudden death is typified by great variability among patients, these recommendations may be overly restrictive for many patients with this disease, and it is possible that a subset of low-risk patients can be identified who may continue to engage in more vigorous exercise activities. This article presents our current understanding and approach to evaluating and advising athletes with HCM.