Background Aortic root dilatation and aortic insufficiency can occur in patients with prior conotruncal defect surgery, the Ross procedure, and connective tissue disease (CTD). Valve-sparing aortic root replacement (VSRR) is an excellent choice for these young patients. We present the outcomes of young patients undergoing VSRR by congenital heart surgeons at a single center. Methods A single center retrospective chart review from April 2008 – April 2021 was performed. Patients with aortic root and valve pathology who underwent VSRR were identified. A total of 49 patients were identified by using the hospital surgical database. Three VSRR techniques were utilized during the study period; aortic valve-sparing root remodeling (Group 1, 7 patients), aortic valve-sparing root reimplantation (Group 2, 32 patients), and a modified root remodeling procedure utilizing a geometric annuloplasty ring (Group 3, 10 patients). Patient characteristics, post-VSRR echocardiogram studies and need for intervention, and survival were analyzed. Results Long-term survival after VSRR is excellent with only one death related to cardiac causes. Valve degeneration remains a concern with 13 patients (26%) requiring subsequent aortic valve replacement over the study period. Risk factors for aortic valve reintervention were the aortic root remodeling technique, mild or greater immediate post-operative aortic insufficiency and higher post-operative mitral insufficiency. Follow-up was significantly longer in Group 1 patients compared to Groups 2 and 3. Conclusions Valve-sparing aortic root replacement is safely performed by congenital heart surgeons in a heterogeneous patient population. Valve degeneration remains a concern and greater than trivial post-operative aortic insufficiency should prompt further attempts at valve repair or replacement.