A 3-year-old boy was being studied by neurologists for suspected neurofibromatosis type 1 as he met at least one major diagnostic criterion (scattered café-au-lait spots). He presented with axial exophthalmos OD for 5 months. His VA with Pigassou test was 1. An MRI was performed and reported as a well-demarcated, homogeneous and isodense thickening of the NO with intact meninges compatible with the diagnosis of glioma of the NO in its intracanalicular and intraorbital portion, thus confirming the diagnosis of neurofibromatosis type 1 as it fulfilled two major diagnostic criteria. Given that he had no lagophthalmos and his VA was preserved, it was decided to have check-ups every 3 months during the first year, and check-ups every 6 months during the second year. Although the criteria for progression of optic gliomas are not universally defined, in this case, 4 years after diagnosis, both the VA and the radiological size of the lesion remained stable, so for the time being we did not consider chemotherapy treatment and monitored the patient every 6 months, with annual MRI.

