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Congenital nasopharyngeal masses are rare and usually benign. We encountered a case of a nasopharyngeal cyst with pituitary tissue in a newborn, causing severe airway obstruction. The mass was removed without complications. Considering the rarity of this anomaly, we described the embryologic origins of ectopic pituitary tissue, discussed the differential diagnosis for nasopharyngeal lesions in the children and emphasized on the physical and radiological findings suggestive of ectopic pituitary tissue. Keywords: Nasopharyngeal mass, Airway obstruction, Ectopic pituitary tissue