Background: To present a rare case of a 2-year-old girl with neurofibromatosis type 1 (NF1) who presented with ptosis of the right upper eyelid along with a tumor in the eyelid.

Methods: A magnetic resonance imaging scan of the orbit revealed a solid tumor located extraconally at the site of the right lacrimal gland. A transcranial orbitotomy was performed.

Results: Histopathological examination demonstrated expanded nerve branches/fascicles cut in various planes in between normal lacrimal gland acini. These findings were consistent with a plexiform neurofibroma presumably deriving from the lacrimal nerve and/or a supraorbital nerve branch.

Conclusion: This is the first case of a plexiform neurofibroma involving the lacrimal gland ever described and the tumor shows similarities with neurofibroma in other salivary glands with a high recurrence rate. Plexiform neurofibromas are frequently seen in patients with NF1 and rarely undergo malignant transformation.