Transesophageal echocardiography (TEE) was performed to rule out infectious endocarditis in a 38-year-old male with no medical history. TEE showed a congenital abnormality of the mitral valve. In A (Online Video 1), we observed a mitral valve opening in an intercommissural view, showing a still image in the center of the ring, 2 inlets and 4 mitral leaflets. The left ventricular short-axis view (A, bottom panel) shows the existence of 2 separate holes (Online Video 2). In 3-dimensional reconstructions (B), the valve anatomy can be seen from the left atrium (B, top panel; Online Video 3) and the left ventricle (B, bottom panel; Online Video 4), confirming the existence of 2 symmetrical holes separated by a continuous raphe, each of which has two leaflets. The functional effect is limited to 2 small jets of mild mitral regurgitation, 1 in each hole, as seen in the color Doppler 2- and 3-dimensional images (C, top and bottom panels; Online Video 5).