Abstract
Cleidocranial dysplasia is a rare disease caused by a defect in the RUNX2 gene on chromosome 6p21, which represents an essential transition factor for chondrocyte maturation and osteoblast differentiation. The patient affected by the genetic anomaly is 8 years old, and there are similar cases in the maternal family. He is followed up by a geneticist, being classified in ICD q78.8 (other specified osteochondrodysplasias) and ICD q74.0 (other congenital malformations of the upper limbs, including the shoulder girdle). The patient started treatment at the University Dental Service complaining of black, white, and brown spots on his teeth. The oral clinical and panoramic radiography characteristics of this patient include fusion, microdontia, and gyroversion. The treatment consisted of filling and sealant. The patient is under continuous supervision and guidance. The clinical and radiographic characteristics of this anomaly are rarely reported in the scientific literature.
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