We report a case of luetic saccular aneurysm of the ascending aorta eroding into the right atrium causing an aorta-to-right atrial fistula. The patient had severe aortic regurgitation, pulmonary arterial hypertension, and congestive cardiac failure. Patch repair of the aneurysm from the aortic side, direct closure of the fistulous opening from the right atrial side, and aortic valve replacement were performed. The patient recovered fully. This case is reported because of its extreme rarity and good surgical result.
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