We describe the case of a 43 year-old woman with chronic headache and diplopia. The neuroradiologic exam showed volume increase and alteration in the bone density of the clivus. She was submitted to surgery and the anatomopathologic evidenced fibrous dysplasia. Other 14 of about fibrous dysplasia involving the clivus are related in the literature. We discuss clinical aspects, image exams, histology and treatment of this rare presentation of the disease.