Fibrous dysplasia is a congenital, nonfamilial, metabolic disturbance that produces 2.5% of all osseous tumors, and more than 7% of all nonmalignant tumors in bone. Involvement of the temporal bone, however, is relatively rare. An 8-year-old girl presented with progressive hearing loss. She had a mass in the left external acoustic meatus and hearing loss in her left ear. Radiological studies revealed a temporal mass. The patient underwent two surgeries in 2 months, and the mass was totally excised. A postoperative neurologic examination revealed left facial paralysis. Histological study identified the mass as fibrous dysplasia of bone. After 5 years of follow-up, there were no signs of residual tumor or recurrence of the disease. When indicated, total excision with extensive reconstruction is the treatment of choice for this type of bone neoplasm.