A 9-year-old boy was first noted to have a heart murmur on the 7th postnatal day. Cardiac catheterization at the age of 4 months showed combined valvular and supravalvular aortic stenosis, bicuspid aortic valve and hypoplastic aortic annulus. Emergency open aortic valvotomy was performed. At the age of 6 years, he had infectious endocarditis which was treated medically. Echo-cardiography at this time showed a 90mmHg pressure gradient across the aortic valve. In August 1992 and in March 1993, ballon valvuloplasties were done but without a significant reduction in the pressure gradient. In July 1993, when the patient was 7 years old, repeated surgical valvotomy and Doty's aortoplasty were performed. Two years later the boy had exertional dyspnea, and a cardiac catheterization showed pulmonary artery pressure of 60/27mmHg, and a 110mmHg pressure gradient across the aortic value. In July 1995, he underwent aortic valve replacement with Konno's aortoventriculoplasty, and a SJM 19mm AHP valve was implanted. One year after surgery, he is without any symptoms. Although the end result was acceptable, earlier AVR with Konno procedure may have spared the child from one extra surgical procedure. Strategic options in the surgical therapy of this condition are discussed.