Slightly more than 30 cases of fibrous dysplasia involving the clivus have been reported in the international literature, primarily in the neurosurgery and radiology literature. In this article we present a series of 4 cases involving patients with clival fibrous dysplasia. In a retrospective chart review, 4 patients presenting to tertiary care centers from January 1, 2006, to January 31, 2008, were identified and their presenting symptoms and radiologic findings reviewed. Based on our literature review, we describe the presenting symptoms of patients with clival fibrous dysplasia and characterize the findings of imaging studies associated with this disorder. All patients in this series had a presenting complaint of headache or cranial nerve deficits, which is consistent with previous reports found in our literature review. Additional presenting symptoms that have been reported include dysphagia and a nontender occipital mass; there was also an incidental asymptomatic finding. Magnetic resonance imaging findings are consistent with those associated with fibrous dysplasia at other sites of the body. We conclude that clival involvement in monostotic fibrous dysplasia may not be as rare as previously perceived.