Intracranial arteriovenous fistulas (AVFs) are rare vascular cerebral lesions composed of one or more cortical-pial arterial feeders directly connected with a single draining vein. They differ from the other AV malformations in that they lack a nidus and are located outside the dural leaflets. Because of high flow and pressure AVFs have a high risk of hemorrhage and if untreated cause death in up to 63% of cases. Treatment can be endovascular or microneurosurgical. We describe an infant with hydrocephalus and raised intracranial pressure and MRI findings of a single dilated venous vessel with a proximal varix in the perimedullary spaces confirmed by CT angiography and DSA that found a single artery-single vein fistula we successfully occluded with GDC coils.