Coarctation of the aorta is a relatively common congenital condition. Most infantile presentations of aortic coarctation are related to the associated congenital cardiac abnormalities leading to congestive heart failure or systemic hypoperfusion. We describe a 4-month-old infant who presented with stridor as the sole manifestation of coarctation. Radiologic studies revealed enlarged innominate artery due to the aortic coarctation that resulted in tracheal compression. After surgical correction, respiratory signs and symptoms completely resolved. This case report describes a unique cause of stridor in newborn infants and discusses the potential for vascular anomalies to result in tracheal narrowing.