Aortic regurgitation (AR) may be caused by valve disease or aortic root anomalies, but it is rare in children without other congenital or acquired heart disease. It is more commonly associated with aortic stenosis, but may also be seen in patients with Tetralogy of Fallot (ToF), D-transposition of the great arteries (DTGA), coarctation of the aorta (CoA), endocardial cushion defect, single ventricle, truncus arteriosus (TA), infective endocarditis, and mitral valve disease. Systemic diseases associated with AR include rheumatic fever, systemic lupus erythematosus, and Takayasu arteritis. AR related to significant dilation of the ascending aorta is seen in patients with Marfan syndrome, bicuspid aortic valve, osteogenesis imperfecta, and rheumatoid arthritis. Significant ascending aortic dilation may, in turn, worsen the AR and lead to aortic dissection.