Fibrous dysplasia is a benign condition that can affect the skull and facial bones and cause a broad spectrum of otolaryngologic conditions. We present the case of a boy with polyostotic fibrous dysplasia with involvement of the temporal bone that was first diagnosed when he was 9 years old. His condition eventually became complicated by cholesteatoma and thrombophlebitis of the left transverse and sigmoid sinuses, and he died of his disease at the age of 19 years. We discuss these and other complications of fibrous dysplasia of the temporal bone and their management.