OBJECTIVE: Twenty cases of fibrous dysplasia involving the clivus have been reported, primarily within the neurosurgical and radiology literature. This case series reviews the presentation of 3 patients with fibrous dysplasia of the clivus and reviews the literature regarding this topic. At the conclusion of this presentation, the participants should be able to: (1) Understand the presenting symptoms of patient with fibrous dysplasia of the clivus (2) Review finding of magnetic resonance imaging characteristic of clival fibrous dysplasia METHODS: The study design is a retrospective chart review and review of the literature. Three patients presenting to a tertiary care center within a metropolitan area within the United States from January 1, 2006 to January 31, 2008 were reviewed. Presenting symptoms and radiological finding were reviewed. The English literature regarding fibrous dysplasia involving the clivus was reviewed. RESULTS: There was a slight female preponderance for fibrous dysplasia involving the clivus. All patients within this series had a presenting complaint of headache or visual complaints. These findings are consistent with previous reports in the literature. Additional reported presenting findings include dysphagia, nontender occipital mass and asymptomatic incidental finding. MRI findings are characteristic of those associated with of fibrous dysplasia in other sites of the body. CONCLUSIONS: There may be a slight female preponderance for clival involvement in monostotic fibrous dysplasia. Clival involvement in monostotic fibrous dysplasia may not be as rare as previously thought. Clival fibrous dysplasia commonly presents with headache. The radiographic findings within the clivus are classic for fibrous dysplasia including ground glass appearance on CT and hypointense lesion on T1 and T2 MRI.