Cleidocranial dysplasia (CCD) also known by the name of Marie and Sainton's disease, is a rare autosomal dominant disease characterized by multiple skeletal and dental anomalies. This case report presents a 21-year-old male patient with short stature, hypoplastic clavicles, patent anterior fontanels, maxillary hypoplasia, hypertelorism, parietal and occipital bossing, retained deciduous and supernumerary teeth. On the basis of clinical and radiographic findings the patient was diagnosed with CCD. The details of the case are reported herein because of the extremely low incidence of this disorder. Early diagnosis is crucial as it helps in better management of dental anomalies to improve quality of life. The dental management of CCD demands a multidisciplinary approach with surgical, orthodontic and prosthodontic expertise to improve function and aesthetics.