Patients with cleidocranial dysplasia commonly present with significant dental problems, such as retention of multiple deciduous teeth, impaction or delayed eruption of teeth and the presence of multiple supernumerary teeth. In fact, the presence of multiple supernumerary teeth is one among the pathognomonic triad for this condition; the other two being partial or complete absence of clavicles, and open sagittal sutures and fontanelles. This case report describes a patient having typical features of cleidocranial dysplasia who had to undergo surgical enucleation and debridement for an infected mandibular cyst. Factors dictating the treatment plan and the current trends in rehabilitation for such a patient have been discussed from a dental surgeon’s perspective acknowledging the shift in management paradigm for this condition over the years.

Keywords: Cleidocranial dysplasia, Delayed eruption, Enucleation, Infected mandibular cyst, Supernumerary teeth