To describe three cases of newborn acute respiratory distress caused by a nasopharyngeal choristoma. The cases presented include the radiological, surgical, histological and historical perspective of this entity. Retrospective chart review of three cases and review of the literature. Three cases of nasopharyngeal choristoma are reported each presenting with neonatal respiratory distress during the first day of life. All cases were assessed initially with cross-sectional imaging (CT or MRI) and subsequently confirmed on nasopharyngoscopy/laryngoscopy and surgically removed. Nasopharyngeal choristoma is a rare congenital nasopharyngeal mass which may present with rapid onset of acute upper airway obstruction by virtue of its relative size and anatomic position.