The first case of ‘a metastatic intraluminal myxoma of the aorta’ is reported. The patient was a 48-year-old man who had already developed metastases to his skin and brain from a left atrial myxoma. Then, his myxoma grew in his leg and intraluminally in the aorta and caused renovascular hypertension. Surgical removal of the tumor could relieve his hypertension, but he finally died 6 months after surgery. The findings at autopsy were described and previous reports on metastasizing left atrial myxomas were also reviewed.