To report a case of neurofibromatosis of the orbit and ocular surface.

A 26-year-old man was admitted complaining of conjunctival mass and exophthalmos. Lisch nodules on the iris were observed in the right eye, and corneal opacity with neovascularization and conjunctival mass were detected in the left eye. Orbital computed tomography revealed diffuse infiltrating facial tumors including the left orbit, suggestive of plexiform neurofibromatosis.

We performed evisceration of the left eye and excision of the left orbital mass. Histopathologic examination revealed neurofibroma which was positive for S-100. No recurrence was detected during a 6-month post-operative period.