Cleidocranial dysostosis is a syndrome defined by some workers as "osteodental" insofar as it presents with variously associated skeletal and dental anomalies. The aetiology, clinical picture and pathological anatomy are analysed, stress being laid on dental anomalies and on dentoskeletal irregularities. Finally, two clinical cases observed in recent years at the University of Bari Odontostomatological Clinic are reported.