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Congenital hyporhinia, also known as partial arhinia, is a very rare congenital abnormality of nasal embryogenesis with unknown etiology. It is commonly associated with other craniofacial anomalies which are thought to be caused by an absent or rudimentary nose. A 3-h-old neonate presented to our facility with hypoplastic nasal pyramid, hypertelorism, microcephaly, and micrognathia, a case of congenital hyporhinia with associated anomalies is presented and the embryology and literature review are discussed.
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