We present a case of primary chordoid meningioma of the lung in a 21-year-old patient with Diamond-Blackfan anemia. The neoplasm was incidentally discovered as a new right upper lobe mass on admission chest radiograph. Computed tomography examination showed a round mass with peripheral nodular enhancement. Pathologic diagnosis was conclusive for the rare entity of chordoid meningioma. To our knowledge, this is the first case showing the radiographic features of an extraskeletal and extracranially located chordoid meningioma.