OBJECTIVE: To present a case of symptomatic fibrous dysplasia showing clinical improvement after surgical treatment. CASE REPORT: A female patient, 27 years, was referred for evaluation at our institution. She complained of headache for 4 years, associated with nasal obstruction and abnormal vision (scotoma), worse on the right. Endoscopy was observed the presence of a solid lesion, occupying the middle third and posterior right nasal cavity, rigid consistency and bleeding to the touch. Front was asked to find paranasal sinus computed tomography with contrast, observing lesion vascularity and low high-density, occupying the ethmoidal sinus and esphenoidal rights, involving the orbital apex, apophysis crest gali and the esphenoidal plane, suggesting fibrous dysplasia of the skull base . Given this situation, we opted for surgical treatment, performed orbital decompression and optic nerve, individualize of the dura mater of the anterior tanks, including the sickle and the reconstruction of anterior skull base at the expense of retail. CONCLUSION: Fibrous dysplasia is a bone lesion, benign and recurrent nature of unknown etiology that can affect any bone. In relation to the paranasal sinuses, the maxillary sinus is the most affected, with the monostotic form the most common. It is characterized by replacement of normal bone by fibrous tissue and trabecular haphazardly. Often surgery is necessary, according to the clinical presentation of disease.