Cleidocranial dysplasia is a rare congenital anomaly characterized by multiple skeletal defects of which partial or complete absence of clavicles, delayed closure of fontanels with presence of open sutures and multiple wornian bones forms a striking feature. The oral manifestations are delayed exfoliation, delayed or failing eruption of the permanent dentition with multiple supernumerary teeth, protruding mandible and mid face retrusion. It is also known as Marie and Sainton's disease, mutational dystosis or cleidocranial dysostosis. A 10-year-old female patient reported to the Department of Pedodontics and Preventive Dentistry, Sathyabama University, Dental College and General Hospital, Chennai, Tamil Nadu with the chief complaint of unerupted permanent teeth. Radiological investigations confirmed the diagnosis of cleido cranial dysplasia and various treatment options are discussed in this article.