Intracranial pial arteriovenous fistula (AVF) is a rare cerebrovascular lesion that has only recently been recognized as a distinct pathological entity. A 41-year-old woman (Patient 1) presented with the sudden development of an altered mental state. Brain CT showed an acute subdural hematoma. A red sylvian vein was found intraoperatively. A pial AVF was revealed on postoperative angiography, and surgical disconnection of the AVF was performed. A 10-year-old boy (Patient 2) presented with a 10-day history of paraparesis and urinary incontinence. Brain, spinal MRI and angiography revealed an intracranial pial AVF and a spinal perimedullary AVF. Endovascular embolization was performed for both lesions. The AVFs were completely obliterated in both patients. On follow-up, patient 1 reported having no difficulty in performing activities of daily living. Patient 2 is currently able to walk without assistance and voids into a diaper. Intracranial pial AVF is a rare disease entity that can be treated with surgical disconnection or endovascular embolization. It is important for the appropriate treatment strategy to be selected on the basis of patient-specific and lesion-specific factors in order to achieve good outcomes.