Craniometaphyseal Dysplasia is characterized by hyperostosis of craniofacial bones, with compression of cranial nerves at the foramina, and by changes in the metaphyses of long bones. The changes in long bones resemble to some degree those seen in pyle's disease, but have cretain differences and significant cranial abnormalities with which differential diagnosis could be made with certainty. An interesting and unusual patient diagnosed Craniometaphyseal Dysplasia was recently experienced by authors. The case report with brief review of literstures in presented.