We report a case of a 49 year-old Japanese woman with a left atrial myxoma complicated by an atrial septal defect diagnosed intraoperatively, which was classified as in secundum defect and was 28x110 mm in diameter. The tumor, which was attached by a pedicle to the posterior wall of the left atrium near the posterior commissure of the mitral valve, was removed together with a small portion of the left atrial wall. The postoperative course was uneventful. The pathologic findings were consistent with the diagnosis of myxoma. Since the defect was functionally canceled by the myxoma, the diagnosis of an atrial septal defect was not confirmed preoperatively even by color Doppler echocardiography and cardiac catheterization. Tumor embolization is one of the critical complications of myxoma. Fortunately, she didn't suffer from either systemic or pulmonary emboli. During surgery of myxoma with atrial septal defect, careful manipulation is needed, especially at caval cannulation so as not to damage the tumor directly. In the cases with atrial septal defect or patent foramen ovale, myxomas are generally situated on the interatrial septum. However, in this case the tumor was located on the posterior wall of the left atrium.