A 43-year-old female patient had an amelanotic single tumor thickening the upper right eyelid and observed since early childhood. Clinically, it appeared as a firm mass, without sharp margins, giving a thickened appearance to the superior eyelid. The ophthalmic clinical examination was normal but some subcutaneous nodules attested to the diffusion of the disease recognized as a limited form of neurofibromatosis 1. Although well tolerated, it gave rise to a cosmetic disturbance and the patient was willing to undergo an excision. A complete surgical excision was performed on the lesions and the pathological study disclosed a typical neurofibroma. Regular observation of the different cutaneous nodules was recommended.