Congenital nasal pyriform aperture stenosis has been described as an unusual cause of neonatal nasal obstruction. Clinical suspicion is based on respiratory distress, cyclic cyanosis, apneas, and feeding difficulties. A bony overgrowth of the maxillary nasal processes is thought to be responsible for this deformity. This anomaly has been reported as an isolated feature or can be associated with craniofacial or central nervous system anomalies. Surgery is indicated in cases of severe respiratory distress, feeding difficulties, and when conservative methods fail.