Introduction: Craniometaphyseal dysplasia is a rare genetic disease marked by progressive thickening of the craniofacial bones and aberrant development of the metaphyses in long bones. As a result of diffuse hyperostosis of the skull base, neurological symptoms associated with cranial nerve compression, such as reduced vision, cranial nerve palsy, and deafness can occur. Craniofacial abnormalities include hypertelorism, frontonasal bossing, broad nasal root, prognathic mandible, and defective dentition. The responsible gene is the ANKH. Several studies report hearing loss in patients with craniometaphyseal dysplasia.