Hypertrophic cardiomyopathy (HCM) is a genetic disease of the sarcomeric contractile proteins. A majority of patients with HCM are limited in terms of functional capacity, and a minority of these patients die suddenly. The main aims of management are symptom alleviation and prevention of sudden cardiac death. In patients with HCM, cardiopulmonary exercise testing provides a much more accurate index of functional capacity than New York Heart Association classification status, and it is useful in assessing symptoms after various therapeutic strategies have been implemented. Exercise testing is also valuable in identifying patients with HCM who are at high risk of sudden cardiac death and is an integral part of the algorithm in risk stratification and delivery of prophylactic therapy. Also, cardiopulmonary exercise testing plays an important role in differentiating HCM from other conditions associated with left ventricular hypertrophy, such as physiologic athlete's heart. Therefore, during the last few years, cardiopulmonary exercise testing has provided insights into the diagnosis, determinants, and mechanisms of exercise limitation in HCM. This understanding aids physicians in targeting therapy and developing new treatment modalities.