Study Design. Retrospective consecutive case series. Objective. To review and analyze clinical presentations and radiological imaging of 326 consecutive patients with spinal dural arteriovenous fistula (SDAVF) from 2 institutions. Summary of Background Data. The clinical presentations of SDAVF are nonspecific. Patients may be initially diagnosed with other spinal diseases. Magnetic resonance imaging (MRI) can reveal spinal cord changes associated with the disorder, but neurosurgeons often overlook these changes. Methods. From 1989 to 2009, 326 patients were diagnosed with SDAVF and treated at Qilu Hospital of Shandong University and the Xuanwu Hospital of the Capital University of Medical Sciences. We retrospectively reviewed the clinical records and radiological images of all patients, and collected and analyzed the related data. Results. Two hundred eighty-two males and 44 females (male/female ratio 6.4:1; mean age, 53.9 yr; SD, 12.1) were included in the study. Fistulas were located at the T7 spinal segment (41, 12.6%), but were more typically found at T5 to L5 (273, 82.5%). The most common initial symptoms were lower extremity weakness (234, 71.8%), sensory disturbance (229, 70.2%), and sphincter disturbance (87, 26.7%). These percentages increased to, 85.6%, 80.8%, and 52.5%, respectively, until patients were properly diagnosed. The mean diagnostic time to SDAVF was 19.9 months (SD, 25.2). Two major changes noted on magnetic resonance images were intramedullary T2-weighted signal hyperintensity (284, 87.1%) and perimedullary dilated vessels (251, 77%). Fistulas were often located outside of the vertebral segments of T2-weighted signal change (P = 0.005). Magnetic resonance angiography and computed tomography angiography of 33 (71.7%) patients revealed perimedullary dilated vessels and precisely located fistulas in 19 (41.3%) patients. Magnetic resonance angiography and computed tomography angiography studies of the perimedullary vessels also led to identification of a second fistula through angiography. Degenerative disc disease and myelitis were the most common misdiagnoses, and the patients were often treated incorrectly. Conclusion. "Worsening" and "symptoms combination" are progression characteristics of SDAVF. Patients should undergo spinal magnetic resonance imaging when they are first suspected to have SDAVF. Magnetic resonance angiography and computed tomography angiography as noninvasive angiography are helpful for diagnosis.