Intracranial and craniocervical junction dural arteriovenous fistulas (DAVFs) with spinal perimedullary drainage are rare, and large cohort studies are lacking. The aim of this study was to describe clinical characteristics and angioarchitecture of these DAVFs, share our treatment experience, and identify risk factors associated with subarachnoid hemorrhage and poor outcomes.A total of 158 consecutive patients treated at our neurosurgical center were retrospectively reviewed. The patients were grouped according to lesion location, and their baseline clinical characteristics, angioarchitecture, treatment strategies, and outcomes were summarized.The patients' mean age was 53.4 years. Most patients were male with 141 patients (89.2%). The most common clinical manifestation was nonhemorrhagic neurological deficits (71.5%), followed by subarachnoid hemorrhage (28.5%). Microsurgery was the most common treatment strategy applied for 112 patients (70.9%), 34 patients (21.5%) were treated with interventional embolization only, and 12 (7.6%) received both interventional embolization and microsurgery. At the last follow-up, there were 122 patients (77.2%) with favorable outcomes (modified Rankin Scale <3). Clinical manifestation of numbness (odds ratio [OR] 4.098, 95% CI 1.491-11.263, P = .006), clinical manifestation of urinary dysfunction (OR 3.991, 95% CI 1.378-11.558, P = .011), and pretreatment modified Rankin Scale ≥3 (OR 19.523, 95% CI 5.066-75.242, P < .001) were significantly associated with poor outcomes.Intracranial and craniocervical junction DAVFs with spinal perimedullary drainage are indeed rare. Specific sign on magnetic resonance imaging is beneficial for accurate diagnosis. The choice between microsurgery or interventional embolization is primarily based on lesion location and angioarchitecture. Early diagnosis, prevention of misdiagnosis, and appropriate treatment are crucial for improved outcomes.Copyright © Congress of Neurological Surgeons 2025. All rights reserved.