This report presents a severe case of Poland-Möbius syndrome with central apnea at birth, ventilator-dependent, and with brainstem calcifications. The newborn had unilateral defect of the right pectoralis muscle, breast, and limb. He manifested bilateral paralysis of the cranial nerves resulting in shallow respiration, apnea, and dysphagia. He finally required tracheostomy and gastrostomy. Maternal history revealed multiple uses of cocaine during the first 3 months of pregnancy. The patient supports the Poland-Möbius combination and the possibility of vascular disruption sequence.