Cleidocranial dysostosis is an autosomal dominant syndrome affecting the calvaria and the bones, clavicula and the dentition. Enamel hypoplasia, cleft palate, persistence of primary teeth, missing or impacted teeth are the significant intraoral findings. In this case presentation, a boy 9 years of age demonstrated macrocephalia, dysmorphic face, persistence of coronal and sagittal fontanellas, bulging of forehead, hypoplastic maxilla and highly arched palate. The instance also displayed clinodactile in both hands an watch glass appearing finger and toe nails. Hypertelorism of the eyes, negative pressure in the fairly big ears and absence of the clavicula are the other complaints. Orthodontic evaluation, as well as other radiographic and clinical findings of the patient indicated further multidisciplinary cooperation.

Keywords: Cleidocranial dysostosis, delayed eruption, maxillary hypoplasia