Resumo (EN)
Cleidocranial dysplasia is a developmental anomaly of the skeleton and teeth. It can be inherited as an autosomal dominant characteristic with high penetrance and variable expressivity. It occurs with equal frequency in both genders and races. The clinical appearance of cleidocranial dysplasia is pathognomonic. The stature is mildly to moderately shortened, with neck appearing long and narrow and the shoulders markedly drooped. Maxillary hypoplasia gives the mandible a relatively prognathic appearance, although some patients may show variable mandibular prognathism due to increased length of the mandible in conjunction with short cranial base. The head and neck features are very variable. Herein, we are described one cleidocranial dysplasia case, in an 84 year female patient, with the dentigerous cyst, which she were not kwon the diagnosis of the her congenital syndrome.