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Right-sided aortic arch is a rare anatomic variation of aortic arch anomaly, which may coexist with or without other cardiac defects. We report an extremely rare case of right-sided aortic arch with a right ductus arteriosus and isolation of the left brachiocephalic trunk, without other associated intracardiac lesions. We describe the successful surgical management of this anomaly in a neonate who had bilateral choanal atresia without DiGeorge syndrome or Down syndrome. However, our patient had clinical signs of hearing loss in the postoperative follow-up period. [ABSTRACT FROM AUTHOR]