Craniocervical junction (CCJ) arteriovenous fistulas (AVFs) are rare. Variability in clinical manifestations and treatment strategies for CCJ AVFs stems from differences in their angioarchitecture. Our study aims to categorize CCJ AVFs based on their angioarchitecture and explore the associated clinical features and treatment modalities for distinct CCJ AVF types.The authors conducted a retrospective analysis of patients with CCJ AVFs treated at a single neurosurgical facility over the past decade. These patients were classified based on the angioarchitecture of their CCJ AVFs. The analysis included an evaluation of angioarchitecture, clinical characteristics, treatment strategies, and outcomes.The study included 155 patients, with a median age of 56 years, collectively manifesting 165 CCJ AVFs. Our classification identified 4 distinct CCJ AVF types: epidural AVFs (19 [11.5%]), dural AVFs (98 [59.4%]), radicular AVFs (33 [20.0%]), and perimedullary AVFs (15 [9.1%]). Further differentiation was applied based on the presence of pial feeders. The predominant fistula location was at cervical-1 (77.0%). Ascending intradural drainage (52.7%) and descending intradural drainage (52.1%) were frequently observed drainage patterns. Patients with dural AVF predominantly presented with venous hypertensive myelopathy, whereas patients with other types of CCJ AVFs showed a higher incidence of subarachnoid hemorrhage (P = .012). Microsurgery was the predominant treatment, applied in the management of 126 (76.4%) AVFs, whereas 8 (4.8%) AVFs exclusively underwent interventional embolization and 25 (15.2%) received a combination of interventional embolization and microsurgical treatment.CCJ AVFs can be distinguished based on the fistula location and the arterial feeders. Currently, microsurgery stands as the preferred treatment strategy for CCJ AVFs, whereas interventional embolization plays a distinctive role in cases with specific angioarchitecture or as a pretreatment measure before microsurgery.Copyright © Congress of Neurological Surgeons 2024. All rights reserved.