The aim of surgery in hypertrophic cardiomyopathy is to reduce the intraventricular obstacle, increase left ventricular compliance, correct mitral regurgitation and other associated lesions, such as coronary atherosclerosis disease or endocarditis. Several surgical techniques have been proposed; myotomy, myomectomy, mitral valve replacement and mitral valve plication. The last mentioned technique combined with myomectomy can be performed safely and may represent an alternative to mitral valve replacement in cases with enlarged and elongated mitral leaflets. We review the natural evolution of the disease and its treatment, emphasizing on the possible mechanism by which surgical treatment may ameliorate the disease.