RESUMO
The McCune Albright syndrome (MAS) is a rare, multi-system disease composed of the triad of polyostotic fibrous dysplasia of bone (PFDB), café-au-lait skin hyperpigmentation, and endocrine disorders. The diagnosis involves clinical, biochemical and imaging findings, with dentistry playing an important role in MAS, since many patients present DFPO in the craniofacial bones, including the maxilla and mandible, and in view of their dental needs, the correct management of these patients is not only an essential but important area to be investigated. This report presents a case of a patient with McCune Albright Syndrome, the behavior of the disease over a period of 10 years and how imaging exams such as scintigraphy and tomography were important for planning the dental treatment of this patient, since they are fundamental allies for identification and evaluation of the progression and/or stability of the disease. Key words:Craniofacial fibrous dysplasia, cone-beam computed tomography, scintigraphy, imaging diagnosis.