Mature glial tissue was seen in the middle ear of a patient with no previous pathologic precedents, and a clinical diagnosis of serous otitis media was made. That anomaly was detected, and its corresponding clinical, roentgenographic, and pathologic findings are described. The results led us to diagnose it as a congenital (spontaneous) encephalocele of the middle ear. To our knowledge, other case of pure congenital encephalocele of the middle ear has been reported.