Primary hypertrophic cardiomyopathy is an isolated genetic heart disease characterized by thickening of the myocardium in the absence of an apparent hemodynamic cause. There are two patterns of the obstruction: static, with a muscle band narrowing the outflow tract of the left ventricle, and dynamic, which implies elongation of the anterior mitral valve leaflet. The key to correct treatment of the condition is understanding of the mechanism behind the obstruction. Myectomy is the gold standard of invasive treatment of obstructive hypertrophic cardiomyopathy; it aims to remove the static component of the obstruction. Another common adidition is the mitral valve surgery, aimed at elimination of the obstruction's dynamic component. This article presents a successful mini-invasive transmitral myectomy and mitral valve replacement in a case of obstructive hypertrophic cardiomyopathy with a damaged mitral valve.