Congenital nasal pyriform aperture stenosis is a rare cause of airway obstruction in the newborn. Immediate recognition and therapy are essential for this potentially life-threatening condition. After initial management, which includes establishment of an oral airway, surgical repair of the stenotic bony inlet has been traditionally considered. We present a long-term follow-up of two patients with congenital pyriform aperture stenosis, who were managed expectantly. Pertinent embryology, clinical presentation, and general treatment strategies for these patients are also discussed.