Orbital ependymomas are rare in the orbit and usually occur secondary to extracerebral extension of an intraventricular ependymoma. The authors present a rare case of orbital ependymoma in a 74-year-old female. The patient was initially diagnosed with intraventricular ependymoma at the age of 13 years that required multiple repeat craniotomies for tumor recurrence. She then developed progressive tumor growth with extension into the bilateral frontal lobes and orbit. The orbital involvement produced binocular diplopia, epiphora, and globe distortion with compressive optic neuropathy. To the authors knowledge, this is the first such report in the English language ophthalmic literature.
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