Enterocystomas of the head and neck are exceedingly rare lesions; only 21 cases have been reported previously. We describe a case of a newborn infant with an enterocystoma of the anterior tongue. All of the previously reported cases and the three theories of embryogenesis are reviewed. Diagnostic evaluation includes computerized axial tomography and radioiodine thyroid scan, particularly if the tongue is involved. Complete surgical excision is the only appropriate therapy and is curative.
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