Ictal piloerection is a rare seizure manifestation and usually reported with temporal lobe epilepsy. There was no specific etiology associated with pilomotor seizures which were described in a wide variety of lesional as well as nonlesional epilepsies. This paper will review the association of piloerection and epileptic seizures and recently recognized important relationship with autoimmunity as a newly described potential etiology. Furthermore at the end of the review we will discuss the etiology, localizing and lateralizing features of our illustrative case examples with pilomotor seizures. We would like also to draw attention to sudden unexplained death in epilepsy in one of our cases with pilomotor aura who had drug-resistant epilepsy associated with hippocampal sclerosis.