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The authors report an observation of dominant generalized cortical hyperostosis with multiple and unilateral involvement of a certain number of cranial nerves. In other members of the family, who present with the same bone disorder, involvement of the cranial nerves was limited to the facial nerve, either uni or bilaterally, with a partially or totally regressive evolution. This cranial nerve involvement, which is classic in the recessive form (first described) of generalized cortical hyperostosis, has until now, never been reported in the dominant form of this disorder.
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