Endocardial fibroelastosis is a disease of obscure etiology and pathogenesis but its gross and microscopic findings have been well known. We have experienced 8-month-old male infant who is diagnosed clinically transposition of great vessel with atrial septal defect. Postmortem examination was performed 96 hours after death. The heart weighed 130gm(normal 35 gm), and was globular in shape. Left ventricle was completely occuluded by aortric and mitral atresia. Left ventricular endocardial surface was pearly white in color and measured up to 2 mm in thickness. Microscopically, left ventricle showed fibroelastic thickening of endocardium. The left atrium was opened to right atrium through patent formen ovale. Aortic arch is very hypoplastic but its main branches and coronary artery were branched out in normal position, and connected with pulmonary artery by widened ductus arteriosus. The other chambers and valvular morphology of heart were free from remarkable findings.