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Background and Importance: Intracranial pial arteriovenous fistula is a recently described matter as a rare vascular malformation occurring in children. It is usually located in the subpial space in the intracranial cavity with a high-flow shunt. Case Presentation: Authors report on a 2-year-old child with intracranial pial arteriovenous fistula and progressive enlargement of head and seizures mimicking progressive obstructive hydrocephalus for eight months. Cranial computed tomography showed a hyperdense midline vein of Galen and enlarged straight sinus associated with obstructive hydrocephalus. Presence of an aneurysm of the vein of Galen was suspected. Conclusion: Authors believe that cranial screening with computed tomography for suspected early diagnosis is of high importance.