Various branch anomalies of the aortic arch have been reported, but cases with separate origins of the internal and external carotid arteries with combined direct aortic arch origin of the left vertebral artery are extremely rare. Herein, we present a rare case of aplasia of the left common carotid artery with separate origins of the ipsilateral internal and external carotid arteries and vertebral artery from the aortic arch in a 10-year-old girl. In addition, we review the embryological development and clinical implications of these anatomical variations.