The localized form of subaortic stenosis shows a spectrum of pathological lesions varing from dis- crete membrane to a thickened flbromuscular collar. Aortic valve is commonly involved late in the pro- cess resulting in regurgitation. Because of the likelihood o the progressive obstruction and aortic regurgitation, early elective oper- ation should be considered for the patient with subaortic stenosis. We experienced a case of localized fibromuscular subaortic stenosis associated with aortic regurgi- tation. Excision of the fibromuscular ridge and septal myectomy-myotomy relieved the subaortic seen- osis. Regurgitant aortic valve was repaired by peeling away the Hbrotic tissue on the cusps and subcommissural annuloplasty at the each commissural area. On postoperative echocardiographic examination, the systolic pressure gradient between left ventricle and aorta decreased markedly and the aortic regurgitation was not detected at all.