Fibrous dysplasia is a benign lesion, congenital, recurrent character and etiology unknown, caused by a bone modeling disorder with constant alterations of the normal bone being replaced by immature fibrous tissue. The lesion may involve one or more bones being the maxilla the facial bone more affected. We report a case of polyostotic fibrous dysplasia in a male patient complaining of increased volume in the right genital region. In this case, Computed Tomography (CT) was the main radiographic examination to demonstrate the extension and radiodensity that the lesion assumes in the craniofacial bones, being therefore of fundamental importance in the surgical planning and in the longitudinal follow-up of the operated patient. The diagnosis was confirmed by anatopathology and the treatment of choice was osteoplasty. This article also aims to review the main clinical, radiological, histopathological, differential diagnosis and Fibrous dysplasia treatment.