Background <p>Piloerection, a physiological response to cold or emotional stimuli, is a rare autonomic manifestation of epileptic seizures. The anatomical correlates and electrophysiological mechanisms of pilomotor seizures remain poorly understood.</p> Methods <p>We conducted a retrospective analysis of 13 patients with pilomotor seizures identified from 8482 individuals monitored at the Epilepsy Center of West China Hospital. Demographics, seizure characteristics, neuroimaging, and neurophysiological findings were analyzed to determine the epileptogenic zones and associated etiologies.</p> Results <p>Most (11/13) of our cases showed temporal lobe origin of seizures, with distinctive ictal encephalogram patterns including rhythmic delta activity. Piloerection was consistently accompanied by other autonomic or psychic symptoms. Most patients responded to anti-seizure medications.</p> Conclusions <p>Pilomotor seizures are associated with temporal lobe epilepsy and are frequently associated with specific etiologies such as autoimmune encephalitis.</p>