Skull base venous fistulas are rare. A 15-year-old boy was stabbed in the left side of his neck, just below the mastoid process. Two years later, he presented with a protruded and pulsating left eye with red sclera. A soft murmur was audible all over his head and neck. Angiography revealed an arteriovenous fistula between the third portion of the vertebral artery (V3) and the vertebral venous plexus, as well as the ipsilateral jugular vein (VVJF).The fistula recruited several arterial feeders and rising the venous pressure along the outflow system of the skull base had led to unilateral exophthalmos.