Fibrous dysplasia is an uncommon benign disorder of unknown etiology. The disease was first described by McCune and Albright in separate publications in 1937. The term, fibrous dysplasia was suggested by Lichtenstein in 1938. The disease has since been found to have 3 different variants: monostotic, polyostotic, and McCune-Albright syndrome. It is a slowly progressive bony disorder where normal bone is replaced by abnormal fibrosseous tissue. Involvement of fibrous dysplasia of the temporal bone is usually unilateral. The squama becomes thickened and the pneumatic system is obliterated. Because fibrous dysplasia shows a predilection for the facial and cranial bone, where it causes deformity and dysfunction. In this paper, we report a case of fibrous dysplasia of the temporal bone. We discuss the characterisitic features of this specific location of the disease, the differential diagnosis, and the treatment policy. We also address the issue of vertigo.