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Fibrous dysplasia (FD) is a rare, benign, chronic, slowly progressive bone disorder characterized by the replacement of normal bone and bone marrow by fibrous tissue, leading to deformity, pain and functional impairment. It was considered a disease of unknown etiology, uncertain pathogenesis and diverse histopathology. It was later discovered that was caused by a non-heritable activating mutation in the a-subunit gene of the stimulatory G-protein coding gene. Temporal bone involvement is the least frequently reported type, especially in children. The purpose of the current manuscript was to report a rare case of fibrous dysplasia of the left temporal bone of a 17-year-old child who came to the emergency room of our hospital with otalgia and progressive hearing loss on the left ear. [ABSTRACT FROM AUTHOR]