In an 82-year-old female case of endocardial cushion defect (ECD), a systolic regurgitant murmur was heard at the apex, and her ECG showed atrial fibrillation without right bundle branch block or left axis deviation. An echocardiogram demonstrated atrial septal defect (ASD) and a cleft of the anterior mitral leaflet with calcification. She died of refractory congestive heart failure. Autopsy revealed ECD (intermediate type) with mitral and tricuspid cleft, and ASD (ostium primum type, 2.0 x 1.0 cm in diameter). In addition, mitral ring calcification and calcification of the cleft mitral valve was disclosed, causing mitral stenosis in addition to mitral regurgitation due to the cleft mitral valve. This was the second oldest Japanese autopsy case of ECD. We concluded that echocardiographic examinations, including color flow imaging, in aged patients with heart murmur are necessary to confirm the diagnosis of congenital heart disease in the aged.