Primary tumors of the heart are rare, three-quarters of the tumors are benign, and nearly half of the benign heart tumors are myxomas. Cardiac myxoma usually originate in the left atrium about 75 percent, but, only 3 to 4 percent of myxoma are detected in the left ventricle. Cardiac myxoma is histologically benign, but may be lethal because of their position. We reported a case of 65-year-old man with left atrium myxoma associated with inferior vena caval thrombi and pulmonary embolism. After the operation of myxoma, the Budd-Chiari syndrome developed and the patient died due to hepatic failure.