Cardiovascular syphilis and syphilitic aortitis, known as late complications of syphilis, are rarely encountered in clinical practice. However, an increase in the number of syphilis cases has been reported in recent years, during which it has also manifested as a complicating infection in those with HIV. A 66-year-old man, who had no previous laboratory findings of syphilis and no subjective symptoms, presented with a complaint of dyspnea. A diagnosis of cardiovascular syphilis was made based on a positive syphilis serological reaction in the preoperative examination for surgical treatment of a left coronary artery ostium obstruction and aortic regurgitation. Three weeks after treatment with amoxicillin, the patient underwent an aortic valve replacement with a bioprosthetic valve and coronary artery bypass surgery. The aortic wall was yellowish with marked circumferential wall thickening and erosion of the intima. The area where the left coronary artery originates was occluded by intimal thickening and revealed only a dimple-like scar. The aortic valve was tricuspidate with thickening and a shortening of the left coronary leaflet. Pathology showed inflammatory cell infiltration of the aortic tunica media, consistent with syphilitic vasculitis. The postoperative course was uneventful and the patient continues to be treated with antibiotics on an outpatient basis.