We report an interesting case of a 66-year-old man with acute myocardial infarction (AMI) with bilateral coronary ostial stenosis cardiovascular syphilis complicated by aortic regurgitation (AR). A 12-lead electrocardiogram and blood tests on arrival suggested AMI, and echocardiography showed moderate AR. Emergency coronary angiography showed bilateral coronary ostial stenosis. The patient underwent emergency surgical treatment, coronary artery bypass grafting, and aortic valve replacement with a bioprosthetic valve. On arrival, rapid plasma reagin and Treponema pallidum hemagglutination tests were 172.2- and 1187.5-fold, respectively. These results suggested cardiovascular syphilis, which was confirmed by pathological findings. The postoperative course was uneventful and the patient was transferred to another hospital on postoperative day 25. This patient received intravenous penicillin for 2 weeks and subsequently oral amoxicillin. When both AR and coronary ostial stenosis are found, it is necessary to consider the presence of cardiovascular syphilis.