Fibrous dysplasia of the temporal bone is a rare condition characterized histologically by proliferation of fibrous tissue with scattered trabeculae of immature bone. Eighteen cases of monostotic fibrous dysplasia of the temporal bone have been reported in the literature. The clinical course of temporal bone fibrous dysplasia is unpredictable. Potential complications include cholesteatoma, recurrence, and malignant transformation. Surgery has been the recommended treatment, but the indications, approach, and extent have not been clearly established. The introduction of computerized tomography with high-resolution bone reconstruction is a significant advance in the therapeutic approach to temporal bone fibrous dysplasia. It accurately defines the extent of the disease within the temporal bone, and periodic scanning will reveal any progression. This information can be used to resolve many surgical dilemmas and to minimize secondary complications. This article includes a comprehensive review of the literature on temporal bone fibrous dysplasia and summarizes a case in which computerized tomography was used.