The temporo-mandibular joint is directly related to the anterior wall of the external ear canal. Therefore, defects in this wall may lead to herniation of the soft tissues of the temporo-mandibular joint into the external ear canal. Such herniation usually is secondary to external injuries, iatrogenic trauma, neoplasms, and so on, but it may also be congenital. We report a well-documented case of temporo-mandibular joint herniation into the external ear canal of probable congenital origin.