Fibrous dysplasia is a benign developmental disorder of the bone. Ten percent of patients with monostotic fibrous dysplasia have craniofacial bone involvement. Twenty-six patients were followed up at the Neurosurgery and Otolaryngology, Head and Neck Surgery Departments between 1990 and 2004. The surgery was performed in 14 patients with considerable cosmetic and functional disturbance. Twelve patients who had mild pain and mass at the site of the lesion were followed up. Cosmetic and functional results, symptom analysis, and frequency of site of involvement were reviewed and compared with previous series. Cranial involvement including frontal, parietal, sphenoidal, and occipital bones was found in 13 (52%) patients. The next common site was the maxillary bone in 9 (33%) patients. Four patients had orbital decompression caused by fronto-orbital and sphenoid bone involvement. None of the patients had any major complication of surgery. Because of its benign nature and decreased growth rate after adolescence, surgical treatment should be reserved for those with functional impairment or severe cosmetic disturbance.