Objectives: To describe the clinical and radiological findings in a consecutive series of patients diagnosed with fibrous dysplasia of the skull. Patients and methods: A retrospective analysis of collected data for 36 patients with histopathologically confirmed fibrous dysplasia involving the skull is presented. The demographic data, clinical presentation, radiographic characteristics, and the management of these patients were reviewed. Results: All 36 patients in this review were diagnosed with fibrous dysplasia involving at least part of the skull. In this study, the most commonly involved area of the skull was the frontal bone (52.78% of patients). The next most common area of skull was the temporal bone (30.56% of patients), followed by the sphenoid bone (25% of patients), the parietal bone (19.44% of patients), and orbital bone (13.89% of patients). The principal clinical presentation included headache, local lump, exophthalmos, visual disorder, cranial nerve paralysis, and facial malformation. These patients were treated by surgical treatment, and several of our patients underwent various degrees of reconstruction to optimize function. Conclusions: Effective surgical treatment may improve the short-term outcome in these patients, and a "tailored" surgical approach is necessary. (C) 2011 Elsevier B.V. All rights reserved.