In a patient with endocarditis and significant aortic insufficiency, two-dimensional echocardiography revealed an abnormal mitral valve configuration with division of the valve into two separate orifices. At autopsy, a double orifice mitral valve with two sets of valve leaflets was observed. Appreciation of this echocardiographic abnormality is important because double orifice mitral valve is associated with other congenital anomalies and this echocardiographic configuration may be confused with other cardiac abnormalities.