Fibrous dysplasia has been frequently reported to involve the spine in the polyostotic form, but only rarely in the monostotic form of the disease. The present case demonstrates monostotic fibrous dysplasia of the fourth cervical vertebra. We present the plain radiographic, CT-scan, scintigraphy and histologic examinations. C3-C4 posterior and anterior arthrodesis was performed. The post-operative follow-up was satisfactory.