Objective To investigate the clinical and radiological features of dural arteriovenous fistula (DAVF) manifested as neurological defects to improve its diagnostic rate.Methods A retrospective analysis was performed on the clinical and radiological data of 3 patients with DAVF manifested as neurological defects.Results Case 1:paroxysmal anepia and right hemiplegia, misdiagnosed as transient ischemic attack (TIA); Case 2:head injury history, dementia, walking unsteadily, and urinary incontinence, misdiagnosed as normal intracranial pressure hydrocephalus; Case 3:9 years of diabetes mellitus, left oculomotor nerve palsy, misdiagnosed as diabetic oculomotor nerve palsy. The 3 cases were diagnosed with DAVF by digital subtraction angiography (DSA). For the first two cases, asymmetric vascular flow voids of bilateral cerebral cortex were found on magnetic resonance imaging (MRI), which suggested DAVF. The head MRI and computed tomography angiography of the third patient with left oculomotor nerve paralysis showed no abnormality, so the DSA of the whole brain was performed to exclude aneurysm. Unexpectedly, it was found that the cause was DAVF of the cavernous sinus region supplied by the external carotid artery system.Conclusions When the patients with neurological defects have mismatched clinical manifestations and imaging findings and unsatisfactory treatment outcome, DAVF should be considered, and the subtle changes in vascular morphology on head MRI should be noticed to reduce misdiagnosis.