DOI: 10.36347/sjmcr.2021.v09i04.019

Pages: 370-372

Neurofibromatosis 1is the most common phacomatosis and neurofibromatosis, with an incidence of 1/2500 births. Transmitted in the autosomal dominant mode, it results in the presence of tumors mainly of the skin, cerebral and peripheral nerves, and in bone and vascular dysplasias. In this presentation, we report the case of a 4-year-old girl, who had consulted for a trailing exophthalmos for two years without any particular pathological history, but the scanner objected to sphenoidal dysplasia with right hemi-megalencephaly, in front of the worsening of her exophthalmos, an MRI was performed with clinical reassessment which demonstrated the appearance of café-au-lait spots with OBNIs and a plexiform neurofibroma on MRI.