Background: Fibrous dysplasia of the skull is difficult to manage when the cranial nerves, skull base, and face are involved. The procedure for the surgical treatment of this lesion needs to be studied clinically because of the low incidence rate and its operative complicacy. Faced with this, combined with our clinical experience, we explore the surgical strategy and operative procedures of the skull fibrous dysplasia. Methods: Twelve patients with skull fibrous dysplasia have been studied with neuroimaging and underwent operative removal of the lesion and cranioplasty and/or reestablishment of the skull base. Results: Excellent operating outcome was obtained in 9 cases and good in 3 based on the evaluation of the lesion removal, cranioplasty, skull base reestablishment, and cranial nerve function recovery. Conclusion: Surgical treatment is suitable for a patient whose lesion damages his or her cranial nerve function and/or appearance. In general, removal of the lesion, cranioplasty, and/or skull base reestablishment can be finished in 1 stage. (C) 2009 Elsevier Inc. All rights reserved.