Due to shortcomings of any valve replacement, repairing and retaining the native valve may be beneficial for congenital aortic valve disease.Retrospective review of data and follow-up of aortic valve repair from a single institution.From 1993 to 2001, 56 patients underwent aortic valve repair [median age 13.4 years (range 1 day to 45 years)]. The predominant aortic valve lesion was mixed aortic stenosis/aortic insufficiency 25 (45%), aortic insufficiency 24 (43%) and aortic stenosis 7 (13%). Repair techniques included sub-commissural plication 36 (64%), commissurotomy 24 (43%), cusp plication 15 (27%), pericardial patch cusp extension 8 (14%) and resuspension of commissures 4 (7%). Most patients (88%) required a combination of techniques; 61% required additional procedures. Hospital survival was 55/56 [98%; (95% CI 91-100%)] no patient was discharged on anticoagulation for aortic valve pathology. Fifty-three patients [95%; (95% CI 85-98%)] remain alive after a median follow-up of 37 months; four survivors required aortic valve replacement and two required repeat aortic valve repair [84%; (95% CI 72-91%) reintervention-free survival].(1) In this study, aortic valve repair for congenital abnormalities avoided reoperation in the majority of patients, avoided anticoagulation and retained growth potential of the valve. (2) Repeat aortic valve repair or replacement was used to treat subsequent valve deterioration.