A 3-month-old girl with "noisy breathing" was found to have situs inversus totalis, corrected transposition of the great arteries [I,D,D], and a vascular ring. The ring was composed of a left aortic arch with normal branching pattern and a right ligamentum arteriosum that extended from a diverticulum off the descending aorta and coursed retroesophageal and to the right to join the pulmonary artery. There was no circumflex component of the aorta or aberrant subclavian artery. The descending aorta was left sided. Compression of the esophagus and trachea was noted on contrast esophagram, magnetic resonance imaging (MRI), and at the time of surgery to divide the vascular ring. In association with her corrected transposition, the patient also was shown to have a mild Ebstein's deformity of the right-sided (systemic) atrioventricular valve and electrocardiographic evidence of Wolfe-Parkinson-White syndrome. The combination of situs inversus totalis, corrected transposition of the great arteries [I,D,D], and an aortic arch anomaly has not been previously reported. In addition, the aortic arch anomaly suggested by MRI imaging and confirmed at surgery has previously only been postulated to exist but to our knowledge never reported.