Children with bicuspid aortic valve (BAV) have aortic dilation that is present and progressive from birth irrespective of the functional state of the valve. There are no published data comparing aortic dilation in children with BAV with and without aortic coarctation (CoA). The objectives of this study were to (1) compare differences in aortic dimensions and rates of dilation between children with isolated BAV with those of children with BAV and CoA and (2) identify risk factors for the development of aortic dilation. Patients with BAV with CoA (group A) and without CoA (group B) were identified from our echocardiographic database (1993 to 2006). Aortic measurements at 4 levels were obtained, and z scores were compared. Criteria for exclusion were severe aortic regurgitation/stenosis, previous aortic valvuloplasty, complex left-sided cardiac disease, ventricular septal defects, and Turner, Noonan, Williams, and Marfan syndromes. There were 600 echocardiograms in 247 patients. Group A had 192 echocardiograms in 53 patients (median age 11.3 years; range 0 to 30; median follow-up 7 years), and group B had 382 in 194 patients (median age 8.7 years; range 0 to 29; median follow-up 4 years). Group B had significantly greater ascending aorta dimensions (p<0.05) and significantly faster rates of aortic dilation (p<0.0001). The ascending aorta in patients with BAV and CoA did not dilate to the same degree as in patients with isolated BAV. In conclusion, valve morphologic characteristics and function and age at CoA repair had none to minimal impact on aortic dimensions.