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The average age is before the end of the first year of life 4 , 5 , 15 and the preferred anatomical sites are the cranio- maxillo-facial skeleton: midline maxillary bone (68%-80%), skull (10.8%), mandible (5.4%) as the case described in the present clinical case, in which the premaxilla and left maxillary portion were affected, extending to the floor of the nostrils