Brain heterotopia of the tongue is a rare, congenital malformation, probably not embryologically related to encephaloceles or to the so called "nasal gliomas." The progressive growth frequently noted confers to the lesion a tumor-like appearance and can raise a clinical suspicion of some rare neonatal neoplasia. The non-neoplastic nature of the lesion is well established, and the growth may well be due to the fact that heterotopic brain grows as rapidly as normal brain in the infant. Although local recurrence after incomplete resection has been reported, the clinical behavior of brain heterotopia of the tongue is benign.