Isolated congenital nasal malformation is rare; the isolated absence of any specific nasal structure is even rarer. They are related to craniofacial stenosis syndromes and to facial cleft, which are described in Tessier classification; also they can appear in 58 complex genetic syndromes. Nasal malformations may be acquired as a consequence of traumas, tumors, infectious diseases, or sequelae of aesthetic surgery. Gorham's syndrome is a rare disease that produces spontaneous and asymptomatic disappearance of any bone of the skeleton. In the world literature, there is no case of Gorham's syndrome with disappearance of the nasal bones. This case report is of a 20-year-old patient who sought correction of an aesthetic defect produced by a cartilaginous hump without the presence of the nasal bones. The absence of both nasal bones is produced by failure of the development of both centers of ossification. Through study of embryological development of the nasal structure, isolated absence of the nasal bones can be explained.
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