Hypertrophic cardiomyopathy (HCM) is a genetic disorder and it is recognized as the most common cause of sudden cardiac death in the young, and an important substrate for disability at any age. Anesthetists may be confronted with clinically unrecognized HCM and must be prepared to anticipate the hemodynamic changes and cardiovascular instability that such patients may impose. When HCM patients are subjected to the stress of surgery, perioperative complications occur and can be devastating. Anesthesia providers need to be aware of the relevant pathophysiology and the mechanisms that may trigger or accentuate dynamic left ventricle outflow tract obstruction. Factors that worsen the degree of left ventricle outflow tract obstruction and hemondynamic strategies to improve cardiac outoput are described in the present review. Strategies to respond to hypotension must be promptly instituted to prevent the development of cardiovascular collapse, and subsequent complications. Therefore, a complete understanding of the pathophysiology, hemodynamic changes and anesthetic implications is needed for successful perioperative outcome.