Neurofibromatosis type I and unilateral ophthalmic artery occlusion

Etiological investigation of a 15-year-old boy with left ophthalmic artery occlusion led us to a diagnosis of neurofibromatosis type I as there were numerous large cafe-au-lait spots, axillary freckling, and brain MRI changes consistent with a hamartoma. In light of the present case, ophthalmic artery occlusion may be a rare feature of neurofibromatosis type I besides more commonly described cerebrovascular changes.