A 6-year-old boy was admitted with infective endocarditis and aortic regurgitation. Clinical signs of infection were severe. The leukocyte count was 13, 100/μl and the C-reactive protein (CRP) was elevated to 17.2mg/dl. Blood culture was positive for Staphylococcus aureus. Echocardiography showed a vegetation 3mm in diameter on the aortic valve, and a perforation of the right coronary cusp with moderate aortic regurgitation. With antibiotic therapy, clinical signs and laboratory data of infection improved at an early stage. We decided to operate after his complete recovery from infection. Laboratory data normalized completely in 6 weeks, but echocardiography demonstrated aneurysmal change of the right coronary sinus and severe aortic regurgitation. The Ross operation was performed on the 44th day. At operation, it was noted that the non-coronary cusp was destroyed completely leaving only strings of fibrous tissue. A perforation of 3mm in diameter was also found on the right coronary cusp. There was a mural aneurysm near the right coronary orifice without abscess formation in the surrounding structure. A pulmonary autograft was transplanted to the aortic root after resection of the destroyed aortic cusps, aortic root and the mural aneurysm. The right ventricular outflow tract was reconstructed using an autologous pericardium as a posterior wall and the Monocusp ventricular outflow patch (MVOP) #22 as an anterior transannular patch. The postoperative course was uneventful. Postoperative echocardiography revealed no aortic regurgitation.