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We report the evolution of an orbital apex tumor that underwent spontaneous regression, followed several years later by rapid growth and loss of vision. A turkey farmer had a right apical lesion, initially thought to be an optic nerve meningioma. The mass spontaneously regressed over 2 years, with marked improvement of vision. Eight years later, the patient presented with loss of light perception and severe proptosis of the right eye from a massive orbital tumor that extended in the cavernous sinus. An eyelid-sparing orbital exenteration was performed. The pathologic diagnosis was diffuse large cell lymphoma. This case demonstrates that non-Hodgkin lymphoma of the orbital apex may resolve spontaneously or it may enlarge rapidly, degenerating into more aggressive forms.