We report the case of a 54-year-old man without previous medical history who presented with sudden vertical diplopia and frontal headache. Clinical examination and Lancaster's test were consistent with superior rectus muscle palsy. Brain CT scan, MRI and CSF examination were normal. Cerebral angiography revealed a dural arteriovenous fistula at the base of the anterior cranial fossa with bilateral arterial supply from small branches of the ophthalmic artery. The causal relationship, the pathophysiology and the therapeutic approaches are discussed.