The authors describe a neonate with unilateral pulmonary agenesis and esophageal atresia (Gross type C). Definitive repair for the atresia was completed during the early neonatal period, and the patient is alive and well more than 1 year after surgery. Most patients with pulmonary agenesis associated with esophageal atresia have not survived, mainly because of postoperative cardiorespiratory failure. Early protection and preservation of respiratory units is essential for the management of these patients.