Paroxysmal neuropathic pain is an uncommon complaint among pediatric patients visiting the emergency department. It is a rare presentation in children with syringomyelia. Patients with syringomyelia may present with a variety of pain symptoms. It is the site and extension of the syrinx, which determines the character of pain. We report an adolescent with Chiari malformation type 1 with syringomyelia who presented with neuropathic pain, dysesthesia, and absent triceps (C7) reflex. The pertinent literature is reviewed.