An extremely rare case of myxomas originating from the mitral leaflets was diagnosed in a 64-year-old man presented with a history of exertion dyspnea and palpitations. Two masses originating from the anterior and posterior mitral leaflets in the left ventricular (LV) cavity, causing LV outflow obstruction, were detected by echocardiography. The myxomas were successfully removed with the mitral leaflets via left atriotomy and mitral valve replacement. No embolic events occurred in the preoperative or postoperative period. In this article, we wanted to present.