Double orifice mitral valve is a rare congenital malformation. Most commonly it is associated with other cardiac diseases, usually in children. The echocardiographic technique has allowed a noninvasive and more frequent detection of this entity. The isolated occurrence of this anomaly is exceptional. We present the case of a 68-year-old male with double orifice mitral valve presented with severe mitral regurgitation secondary to chords rupture, without any other congenital malformations.