Congenital mitral valve disease is a rare form of mitral regurgitation. The etiological diagnosis is often challenging. The transthoracic echocardiogram is presented as a good initial approach method. The case of a 29-year-old patient referred for the diagnosis of severe congenital mitral regurgitation in parachute is presented. This report aims to illustrate the clinical and echocardiographic presentation of congenital mitral regurgitation.