Background We reviewed our experience with valve-sparing aortic root reconstruction (VSARR) using the sinus of Valsalva graft in children, teenagers, and young adults with connective tissue disorders. Methods Results of a single-center experience with VSARR in children, teenagers, and young adults were retrospectively analyzed. End points were death, freedom from reintervention, and freedom from valve dysfunction. Results Between 2003 and 2010, 16 patients (Marfan, 9; Loeys Dietz syndrome, 6; conotruncal, 1) underwent VSARR. The mean age was 20 ± 7.4 (range, 9 to 36 years). Indications for VSAAR were aortic root enlargement in 14 (sinus of Valsalva Z-score, 6.2 ± 2) and aortic dissection in 2. Additional procedures included replacement of the ascending aorta in 7, with additional replacement of the aortic arch in 2. No early or late deaths occurred. One patient required a pacemaker. One patient with Loeys-Dietz syndrome required reoperation for aneurysmal dilatation of the coronary buttons. Two patients underwent replacement of the thoracoabdominal aorta for chronic dissection. Follow-up by echocardiography or magnetic resonance imaging at a mean of 33 ± 29 months showed more than mild aortic regurgitation in 2 patients. Both patients with moderate aortic insufficiency also had a bicuspid aortic valve. Conclusions VSARR using the sinus of Valsalva graft is a reproducible technique that achieves acceptable early and intermediate results. It is suitable for children, teenagers, and young adults. Anticoagulation is avoided. The procedure is appropriate for emergency operations but should be used with caution in patients with a bicuspid aortic valve.