Monostotic fibrous dysplasia of the temporal bone

Fibrous dysplasia is a rare but interesting benign condition of the bones which affects either a single bone or multiple bones, with or without endocrine abnormalities. Only 75 cases affecting the temporal bone have been reported in the world literature, and to these we add another case. Although systemic involvement in the form of endocrine abnormalities and skin pigmentation has been reported in the past, no case has yet been reported with a congenitally absent kidney in the monostotic form of the disease.