We have experienced a case of plexiform neurofibrorna of the right upper eyelid and orbit in a 12-year-old girl who had typical skin features of neurofibromatosis and no family history. The non-pulsating proptosis of the right eye and diffuse thickening with hypertrophy of the upper lid, had increased insiduciusly since the birth on. Biopsy taken from eyelirl lesion showed the features of plexiform neurofibroma. Skull X-ray and brain computerized tomogram showed that the right orbit was wider, with the enlarged mass and defects in orbital roof and lesser and greater wings of the sphenoid bone. The surgical excision of the right eyelid lesion was performed.