We describe the clinical and histologic features of the first case, to our knowledge, of hemangioblastoma of the orbit arising from an extraocular muscle. We examined a 73-year-old woman with conjunctival injection, 2 mm of proptosis, and limitation of abduction of the right eye. A medial-lateral orbitotomy was used to approach the medial rectus enlargement noted on orbital imaging. Histopathologic examination with light microscopy and immunohistochemistry demonstrated the characteristic findings of a hemangioblastoma.
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