Congenital Nasal Pyriform Aperture Stenosis (CNPAS) is an extremely rare cause of
nasal airway obstruction. Clinically, it mimics choanal atresia in neonate. It needs
to be differentiated because management differs from each. Pyriform aperture is
located at the most anterior part of the nose and is always the narrowest region
of the nasal cavity. Therefore, nasal obstruction can easily occur if there is slight
decrease in its cross sectional area. CNPAS rarely presents alone. Usually, it arises
together with a midline developmental defect such as holoprosencephaly or
pituitary defect. We present the first ever reported case of CNPAS, in association
with Trisomy 8 Mosaicsm (T8M).