DOI: 10.36347/sjmcr.2021.v09i09.004

Pages: 836-839

Neurofibromatosis (NF) is a neurocutaneous disorder, which involves many organs in the body. There are two types: NF-1 and NF-2. Orbital manifestation is a rarity in NF-1, and it involves dysplasia of the sphenoid bone resulting in herniation of the temporal lobe and subarachnoid space into the orbit culminating in exophthalmos. In this presentation, we report the case of 18 years old boy, admitted for exophthalmosis dating back to the age of 5 years. we performed a cerebral and oculo-orbital MRI which has objectified a sphenoid agenesis (confirmed by CT scan) associated with a neurofibroma of the external canthus of the right eye, we have also objectified multiple signal abnormalities of cerebral white matter corresponding to FASI (focal areas of signal intensity), a more detailed clinical examination was carried out a posteriori which revealed multiple café-au-lait spots, all of these lesions suggestive of neurofibromatosis with cerebral and spheno-ocular involvement.