Pial arteriovenous fistula (AVF) is a rare vascular lesion, with less than 120 reported cases in the English literatures (Hoh et al., Neurosurgery 2001;49(6):1351). The angio-architecture, clinical course and therapeutic options are all different from arteriovenous malformation (AVM), dural AVM or other intracranial vascular lesions. A review of literatures to analyse the clinical course of pial AVF was carried out. The presence of varix dictates the clinical course and presentation. Paediatric type had high percentage of varix, and mass effect as clinical presentation while the adult type usually manifest by haemorrhage. Disconnection of direct shunting, either by endovascular or surgically, is sufficient to achieve successful treatment; therefore, total resection of the lesion is unnecessary.