We have described a primary esophageal meningioma (MG) clinical case diagnosed in a 62-year-old woman; also, we review the literature about extracranial MGs. To our knowledge, this is the first case report of an extracranial MG occurring primarily in the esophagus. These are benign neoplasms reported classically in the central nervous system (CNS). The extrancranial MGs have histopathologic and inmunohistochemical features identical to those observed in CNS MGs; thus, the main diagnostic hurdle is to keep it in the differential for lesions occurring outside the CNS.