A classic neurilemmoma was found in the eye of a 31-year-old man with known neurofibromatosis. The pathologic features of this rare intraocular tumor were examined histologically, immunohistochemically, and ultrastructurally. Contiguity of the neurilemmoma with its nerve of origin, the long posterior ciliary nerve, was clearly demonstrated. Expansion of the nerve by proliferating Schwann's cells was present.