Extra-axial ependymoma of the cerebral convexity: A very rare intracranial adult tumor

Background Ependymomas are rare adult tumors that originate from the ventricular system or the ependymal surface of the central canal. Extra-axial supratentorial ependymomas are extremely rare, and only five cases have been reported to date in the English literature. Case presentation A 46-year-old previously healthy male presented with a gradual painless loss of vision in the right eye. Cerebral MRI showed a right parietal-occipital lesion resembling an atypical meningioma. Surgical resection was performed, and immunohistochemical staining results concluded that it was a very uncommon location of a grade 3 ependymoma. Favorable outcome was observed one year after completion of postoperative radiotherapy. Conclusion Clinical and imaging aspects are misleading in rare brain tumors, and immunohistochemistry is essential to re-address diagnosis.