Fibrous dysplasia is a benign fibro-osseous disease of bone of unknown etiology. Its occurrence in the craniomaxillofacial skeleton is frequent and varies in severity from an asymptomatic monostotic lesion to polyostotic involvement resulting in progressive functional deficit and aesthetic problems. With the advent of refined instrumentation and craniofacial surgical techniques, a more aggressive, non-disabling approach to these benign yet deforming fibro-osseous growths is possible. In some patients, complete excision of the involved bone with graft reconstruction of the resultant defect with primary autogenous bone may be possible. Lifelong continuous ongoing monitoring of the involved region is required throughout the patient's life.