The congenital stenosis of pyriform aperture is an unusual cause of neonatal nasal obstruction. It is due to bony overgrowth of the nasal lateral process of the maxilla. Initially this narrowest part of nasal airway was considered an isolated deformity; subsequently the congenital Stenosis of pyriform aperture was thought to represent a microform of holoprosencephaly. In this report a male neonate had respiratory distress, cyclic cyanosis and apnea after delivery. The patient underwent surgical correction of pyriform stenosis by sublabial access. In the follow up, the patient had good evolution. The report of this deformity shows an important cause of neonatal nasal obstruction and its differential diagnosis with bilateral choanal atresia. Congenital stenosis of nasal pyriform aperture can be surgically corrected when necessary.