A 40-year-old white woman had slowly progressive unilateral loss of visual acuity and increasing proptosis during an eight-year period. Ophthalmoscopy and fluorescein angiography revealed chronic disk edema and optociliary shunt vessels in the right eye. Polytomography showed an enlarged right optic canal. Cerebral arteriography demonstrated a dumbbell-shaped tumor blush in the right juxtasellar region and a diffuse tumor blush in the right orbit. Right frontal craniotomy and orbital exploration showed a cranio-orbital junction (spheno-orbital) meningioma that invaded the sclera and peripapillary choroid. Anomalous optociliary veins were demonstrated histologically at the optic disk.