Introduction: Cleidocranial dysplasia (CCD) is a rare dominantly inherited autosomal bone disease that is characterized by delayed closure of fontanelles, presence of open skull sutures, hypoplastic or aplastic clavicles, supernumerary teeth, delayed eruption and impaction of permanent dentition, morphologic abnormalities of the maxilla and mandible, wide pubic symphysis, short stature and a variety of other skeletal changes. CCD is also known as Marie–Sainton disease, mutational dysostosis, and cleidocranial dysostosis. It is caused by mutations of the transcription factor RUNX2, which is known as a major regulator of bone differentiation.

Case report and treatment: In this case report, treatment of a 23 years old CCD patient with multiple impacted teeth and Class III craniofacial relation is presented. Impacted teeth were exposed with surgical procedure and forced to erupt with help of orthodontic elastics then preorthognathic dental alignment were made by orthodontic treatment. Class III craniofacial relation was treated with orthognathic surgery.

Conclusion: Dental abnormalities are a well-known complication that causes morbidity in CCD patients and management of these are performed by a team approach with the overall goal to provide an esthetic facial appearance and functional occlusion. Orthognathic surgery combined with the orthodontic therapy to correct mid-face hypoplasia is considered to reduce the Class III malocclusions for the CCD patients.

Conflict of interest: None declared.