We report here a surgical case of sinus of Valsalva and right atrium fistula associated with acute infective endocarditis (AIE) without perivalvular abscess cavity or aneurysm of the sinus of Valsalva (ASV). A 51-year-old man, who had been given a diagnosis of rheumatic aortic stenosis and regurgitation (AsR) and mitral stenosis and regurgitation (MsR) and tricuspid regurgitation (TR) by echocardiography, had a high fever 2 months after removal of teeth and AIE was diagnosed. He was referred to our hospital because sinus of Valsalva and right atrium fistula were detected by echocardiography and congestive heart failure (CHF) deteriorated during medical treatment. Perivalvular abscess cavity and ASV were not detected by preoperative echocardiography. Medical treatment was continued after admission, and operation was done after amelioration of the CHF and infection were recognized. The aortic valve was removed together with vegetation, two areas of the aortic wall in which the tissue was fragile were cauterized by electrocautery, patch closure at the sinus of Valsalva was performed using a partial of e-PTFE graft and aortic valve replacement (AVR) and mitral valve replacement (MVR) were done. Though residual aortic-right atrium shunt was detected after the operation, the postoperative course was good with no CHF or signs of infection.