Neurofibromatosis type 1 (NF1) has been infrequently associated with different cerebrovascular conditions that may lead to either ischemic or hemorrhagic stroke. Intracranial dural arteriovenous fistulas have not been described in NF1 patients. In this paper we present a unique case of an 8-year-old child with florid NF1 that presented a greater sphenoid wing dural arteriovenous fistula draining directly through the superior ophthalmic vein and causing exophthalmos. The fistula was cured by direct trans-superior ophthalmic vein approach, by means of detachable coils. Only a few cases of dural fistulas in the lesser sphenoidal wing region have been described in the literature but a fistula of the greater sphenoid wing has not been previously described.