Fibrous dysplasia is a slowly progressive, benign disorder of fibro-osseous tissue of undetermined etiology. Two forms of fibrous dysplasia are recognized : monostotic form and polyostotic multifocal form. Involvement of the skull is not uncommon in polyostotic form, and the maxilla and mandible are frequent site of lesion in monostotic form but rarely in the temporal bone. Authors are reporting a case of monostotic fibrous dysplasia of the temporal bone with a review of the related articles.