A pial arteriovenous fistula (AVF) is a rare intracranial vascular lesion characterized by the presence of one or more arterial feeders that are directly connected to its venous drainage without an intervening nidus, which is the hallmark of arteriovenous malformations (AVMs). Previously, these lesions were considered as a subtype of AVM and were often referred to as fistulous AVMs; however, due to differences in angioarchitecture, clinical course, and therapeutic options compared to classic AVMs, dural AVFs, or other intracranial vascular lesions, pial AVFs are now recognized as a distinct entity. In young patients, these lesions are more likely to present with an angiographic varix and symptoms related to shunting effects. In contrast, adults are less likely to present varices, and hemorrhage is the most common form of clinical presentation. Additionally, spontaneous regression is thought to be rare. A 68-year-old man with a history of dyslipidemia and prostatic disease was admitted to the emergency department after insidious onset of progressive headache associated with vomiting and malaise. Neurological examination revealed speech impairment, moderate right facial weakness, and severe right arm weakness. Admission head CT scan showed a medium-sized cortico-subcortical left frontal lobar intracerebral hematoma, and contrast-enhanced head CT failed to reveal signs of abnormal enhancement at the site of the hemorrhage. The patient was admitted to the neurosurgical intensive care unit (ICU) and treated with analgesics, antihypertensives, and antiepileptic prophylaxis. He remained stable, and after 6 days, brain MR angiography (MRA) still failed to demonstrate any associated vascular abnormalities. However, digital subtraction angiography (DSA) performed 1 week after admission revealed the presence of a superficial left-sided frontal pial AVF characterized by a direct connection between the paracentral artery, a branch of the pericallosal artery, and a compressed frontal cortical vein. No intervening nidus or aneurysms were found, and the lesion seemed compressed and deformed by the mass effect produced by the hematoma. The patient was managed conservatively and consequently discharged to a rehabilitation facility. Eight weeks after the ictus, the patient was readmitted for a follow-up angiographic study, which showed spontaneous thrombosis and exclusion of the fistula. This finding was later confirmed by a third angiography performed 6 months later. This chapter discusses the characteristics and management of pial AVFs, including their definition, prognosis, and our unusual experience with documented spontaneous regression.