The manifestations of neurofibromatosis (NF) in the ocular adnexa consist of plexiform neurofibroma in the eyelids, often with orbital extension, and sphenoid wing hypoplasia. NF may be associated with tumours such as optic nerve gliomas, meningiomas or schwannomas. It is the most common peripheral neural tumour of the ocular adnexa causing hypertrophy of periocular tissues, resulting in mechanical ptosis with a characteristic S-shaped deformity in the upper eyelid. Individualized treatment plan must be drawn after proper preoperative assessment, taking into consideration the disfigurement and functionality that a patient.
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