A case of a giant intracranial varix arising from an arteriovenous (AV) fistula is reported. This 16 year old boy presented with a sudden onset of headache and dizziness. The patient had suffered from intermittent seizure attacks since the age of 4. Brain computed tomography (CT), taken 2 years prior to admission, showed compression of the quadrigeminal plate by a suspicious mass lesion. His brain CT, magnetic resonance imaging (MRI), and cerebral angiography on admission demonstrated a giant varix with evidence of haemorrhage in the cerebellum. The varix was fed from branches of the posterior cerebral artery and drained through a dilated vein into the vein of Galen. It was resected surgically. Surgery and/or endovascular obliteration of the AV fistula is required because the varix which develops from an AV fistula can enlarge and rupture.
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