We report the findings of three-dimensional (3D) transesophageal echocardiography (TEE) in a patient with a parachute mitral valve (MV) accompanied by aortic valve (AV) malformation. The results indicated an enhanced echo in MV anterior leaves, incrassate, and shortened subvalvular chordae tendineae, and posteromedial papillary muscle that had echo reinforcement, calcification, retroposition, and a significant decrease compared with anterolateral papillary muscle. In addition, the anterolateral papillary muscle was huge, with the bilateral papillary muscles fused partly, and the posterior subvalvular chordae tendineae incrassate, shortened, and attached parachute-like to the anterolateral papillary muscle. The MV appeared dome-shaped for the open limit in diastole with an MV area of 1.6 cm. Moreover, the left ventricle increased in size and the bicuspid AV was malformed. Continuous wave Doppler angiograph showed that the flow rate increased to 398 cm/seconds at the AV orifice area. A 3D form of the MV structure was observed from the left ventricle using 3D-TEE inspection. The anterolateral papillary muscle was fused with its posteromedial homologue. The chordae tendineae was attached to the anterolateral papillary with the parachute-like structure, indicating dome movement. Copyright (C) 2012, Elsevier Taiwan LLC. All rights reserved.