ABSTRACT
Pyknodysostosis is a rare sclerosing bone disorder that has an autosomal dominant trait. It is characterized by short stature, brachycephaly, short and stubby fingers, open cranial sutures and fontanel, and diffuse osteosclcrosis, where multiple fractures of long bones and osteomyelitis of the au arc frequent complications. A case is presented which has the characteristic oral and radiological findings that are connsistent with this syndrome, along with a brief review of the literature. As oral manifestations and appearance characteristics seen in this disorder arc reliable signs for its further examinations, knowing about these signs would be helpful in the diagnosis as well as the improvement of its prognosis. Accordingly, by knowing the oral symptoms and appearance characterstics of the disease, dentist could be the very first person who would look for the necessary examinations led to the diagnosis of the disease