Pterygium usually has a benign course extending to a limited extent into the nasal cornea. It has not been described in association with systemic diseases, but is more prevalent in equatorial regions and is probably related to ultraviolet radiation exposure and to dry and dusty climates. We describe an atypical recurrent pterygium that extended over the cornea through the inferior cul-de-sac. The atypical appearance and course led us to search for an associated systemic disorder. Physical examination revealed findings of type 1 (peripheral) neurofibromatosis.