A 10-year-old girl presented with painless progressive proptosis of the left eye. CT and MRI of the orbits showed a large ethmoidal mass invading the orbit. Clinically, despite its size, the tumor was not inducing any functional signs. A coronal approach was used to remove the tumor en bloc. Histopathology revealed that the lesion was a chondromyxoid fibroma, a neoplasm unreported in the ophthalmic literature.
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