In a 4-year period from July 1984 to July 1988, 21 children required surgical treatment for subaortic stenosis. Age at operation ranged from 12 months to 17 years (mean, 7.3 ± 8.4 years). Associated anomalies were common and occurred in 13 patients (62%), including aortic valve insufficiency (eight), and stenosis (two), mitral valve stenosis (two) and insufficiency (one), and pulmonary valve stenosis (two) and absent pulmonary valve syndrome (one). A ventricular septal defect was present in five patients, and coarctation and patent ductus arteriosus occurred in three patients each. One patient had a complete atrioventricular septal defect, and one had an aortopulmonary window. Six patients had 15 previous procedures. The left ventricular to aortic gradient ranged from 20 to 170 mm Hg (mean, 59 ± 43 mm Hg). The operation consisted of a transaortic resection (17), transventricular septal defect resection (two), a modified Konno procedure or septoplasty with preservation of the aortic valve (one), and a combined aortoventriculoplasty with homograft reconstruction of the ascending aorta and coronary reimplantation (one). There were no early deaths. One patient required pacemaker insertion for heart block. Follow-up range from 1 to 48 months (mean, 26.4 ± 11.6 months). There was one late death 2 years after operation in the child with the aortopulmonary window who had Heath Edwards Type IV changes on a lung biopsy specimen at the original operation. One patient required reoperation for a residual gradient of 34 mm Hg 1 year after resection. Three patients still have aortic insufficient. No cases of iatrogenic aortic valve injury occurred. Patients with subaortic stenosis represent a heterogeneous group. Early resection and additional procedure can be performed with a low mortality rate and can eliminate aortic insufficiency in many cases.