Background: Spinal dural arteriovenous fistula (SDAVF) is a rare spinal vascular disease. The clinical misdiagnosis rate is very high. The highest misdiagnosis rate is reported in orthopedics. The specific reason for misdiagnosis remains unclear. Objective: To investigate the clinical and imaging manifestations of SDAVF, analyze the causes of misdiagnosis, propose countermeasures, and improve the orthopedists and other specialists’ understanding of this disease. Methods: The clinical data, diagnosis and treatment of 12 patients who had SDAVF that was misdiagnosed as a different disease were retrospectively analyzed, and the modified Aminoff-Logue Disability Scale (ALS) scores before and during follow-up were compared. Results: From 2014 to February 2019, 15 patients were diagnosed with SDAVF at our institution. Twelve (80%) were misdiagnosed; of these, 6 (50%) were misdiagnosed more than twice, and 6 patients (50%) were misdiagnosed at least once. The initial diagnoses included lumbar spinal stenosis and lumbar disc herniation (LDH) in 6 patients, cervical spinal stenosis in one patient, benign prostatic hyperplasia (BPH) in one patient, myelitis in 2 patients, and syringomyelia in one patient. After the initial diagnosis, one patient was misdiagnosed with LDH, one was misdiagnosed with subacute combined degeneration of the spinal cord, and 4 were misdiagnosed with myelitis. The clinical manifestations mainly included weakness and numbness of the lower limbs, urinary symptoms, and numbness of the perineal area. In the 12 misdiagnosed patients, magnetic resonance imaging(MRI) showed signs of spinal cord edema and typical or atypical flow-void patterns. One patient had undergone extended cervical decompression and lumbar decompression. All patients eventually underwent microsurgical treatment. The average follow-up duration was 0.9 years. The modified ALS scores showed significant improvement in gait, bladder function and bowel movement, and the differences before and during follow-up were statistically significant (P<0.05). Conclusion: When patients, especially those with bladder and bowel dysfunction, have symptoms such as weakness of the lower limbs and/or numbness, a diagnosis of SDAVF should be considered. Furthermore, if MRI shows signs of spinal cord edema and typical or atypical flow-void patterns, the diagnosis of SDAVF should be strongly considered, and further angiography is needed to confirm the diagnosis.