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Apertura pyriformis stenosis in the newborn. Respiratory distress in the newborn can have a variety of aetiologies, the best known of which are cardiac and pulmonary diseases. Major nasal airway obstruction is probably often overlooked when acute desaturation of the neonate requires reanimation procedures, although it is well established that the baby is an obligate nose breather at birth. Nasal airway stenosis or atresia could account for a number of unexplained deaths in the delivery room. In the differential diagnosis of major nasal airway obstruction in the newborn, choanal atresia is by far the most common aetiology. However, a few cases of pyriform aperture stenosis have been reported. One child presenting this pathology was recently treated at the Brussels University Children’s Hospital. Unexpectedly, the baby survived until 3 months without any medical support but had severe feeding problems. The diagnosis was confirmed by naso-sinusal CT scan. Surgery was performed at 4 months through an unusual endonasal approach which seems to be less traumatic than the classical sublabial approach. The post-operative course was satisfactory. The 9- month-old patient does not now show residual breathing problems. The presentation will focus on this unusual case.