We report two neonates with distal ductal origin of the left pulmonary artery who also had rare vascular rings comprised of a left aortic arch and right arterial duct with a midline/rightward descending aorta. To the best of our knowledge, this association has not previously been described, although other abnormalities of the left pulmonary artery in the setting of vascular rings have been reported. We review the embryology, utility of imaging, and clinical course.