Primary tumors of the heart are rare entities. We report a unique case of a young woman presenting a left ventricular myxoma, which manifested itself as coronary artery disease and was diagnosed by echocardiography. Emergency surgical treatment was indicated, done through a transeptal approach with extensive resection of the tumor, its pedicle and the papilar muscle to which it was attached as well as the anterior mitral leaflet, which was thinned and deformed by the presence of the mass. Mitral valve replacement was mandatory. A complete retrospective review of the literature is presented.