Aortic valve replacement in the pediatric population is complicated by the often complex nature of the left ventricular outflow tract obstruction. Techniques to enlarge the annulus frequently are necessary. From 1977 to 1991, 32 children underwent an annular enlargement procedure at The Hospital for Sick Children, Toronto. During this same era, 110 children underwent a total of 138 aortic valve replacements. Eleven had the annulus enlarged with a posterior patch technique and implantation of a valve (mechanical 8, porcine heterograft 2, homograft 1) ranging from 20 to 25 mm in diameter. Twenty-two children had an anterior annular enlargement (aortoventriculoplasty) and aortic valve replacement with a valve (mechanical 8, porcine 2, homograft 12) 12 to 27 mm in diameter. One child had a posterior patch enlargement performed, followed by a second operation involving anterior annular enlargement. There was one early death in the posterior annuloplasty group and one late death due to failure of a bioprosthetic valve. There were five hospital deaths in the anterior annuloplasty group (22%; 70% confidence interval [CI], 14% to 32%) and two late deaths. Actuarial survival for the 32 children was 78% (70% CI, 70% to 86%) at 5 years and 65% (70% CI, 48% to 82%) at 10 years after repair. Younger children (age less than 1 year) had a significantly worse survival at 5 years (33%; 70% CI, 14% to 52%) than older children (88%; 70% CI, 82% to 95%). The survivors are well, and no reoperations have been necessary because of the children's outgrowing their valve.