Cleidocranial dysplasia (CCD) is a rare congenital deformity inherited as an autosomal genetic trait with the prevalence of 1:1,000,000. It is characterized by dental defomities such as retained primary teeth, presence of supernumerary teeth, skeletal dicrepancy, malocclusion and retarded/absence of eruption of permanent teeth. The orthodontist take part in the team for patients with CCD to resolve the eruption delay of permanent teeth and correct skeletal discrepancies. The aim of this review is to discuss the history, genetic backgrounds, clinical and dental features, different dental treatment approaches and orthodontic management of CCD. (Turkish J Orthod 2015;28:31-37)