Cases involving intracranial dural arteriovenous fistulas (AVFs) with spinal perimedullary venous drainage exhibit variable presentations, which results in delayed diagnoses. We describe a case of a 66-year-old female with a transverse-sigmoid sinus dural AVF with spinal perimedullary venous drainage who developed dysesthesia and hypalgesia that ascended from the peripheral lower extremities. Sixty cases of intracranial dural AVFs resulting in myelopathy have been reported, and an absence of brainstem signs significantly correlated with a delay in diagnosis (positive group: 3.4 months vs. negative group: 9.6 months, P < 0.05). Intracranial dural AVFs with brainstem signs should be diagnosed without delay because the myelopathy and bulbar symptoms could progress aggressively without alternative drainage routes besides the perimedullary veins. We emphasize that intracranial dural AVFs should be considered as a differential diagnosis in case presenting with symptoms, such as atypical dysesthesia and hypalgesia ascending from the toes, without brainstem signs. Moreover, we should perform cerebral angiography as early as possible because dural AVFs with slow-flow venous drainage can produce false negatives on magnetic resonance angiography.