We report here on a case of double-orifice mitral valve with mitral regurgitation in a 75-year-old female who had complaints of mild dyspnea. Transthoracic and transesophageal echocardiography showed two orifices that were supplied by their own chordae from a different papillary muscle. Color Doppler echocardiography revealed moderate to severe mitral regurgitation due to the flail posterior leaflet of the anterolateral orifice. Except for the persistent left superior vena cava, no other congenital anomaly was demonstrated. The patient became asymptomatic with the administration of angiotensin-converting enzyme inhibitor and diuretics, and she has been scheduled for long term follow-up.