〔Abstract〕 Objective To analyze the clinical features of guillain-barre syndrome (GBS) with headache. Methods 4 patients with GBS complicated with headache who were admitted to Shenzhen Second People's Hospital and Peking University Shenzhen Hospital from March 2017 to October 2019 were selected to retrospectively analyze their clinical data and summarize their clinical characteristics. Results The main symptoms of 3 patients were headache accompanied by fatigue and limb numbness. 1 patient had headache accompanied by double vision. Craniocerebral computed tomography (CT) and craniocerebral magnetic resonance imaging (MRI) examinations were performed in 4 patients: no clear abnormalities were found, and lumbar puncture cerebrospinal fluid examination indicated protein-cell separation. Electromyography examination in 3 patients suggested multiple impaired peripheral nerve motor conduction function. Brain stem auditory evoked potential (BAEP) examination in 1 patient suggested bilateral central segment damage (from intracranial segment of auditory nerve to central segment of brain stem), and binocular visual evoked potential (VEP) suggested impaired conduction function of visual pathway. Headache symptoms were relieved after intravenous immunoglobulin and hormone therapy in all 4 patients. Conclusion Headache is a rare complication of GBS, which is easy to identify as migraine or intracranial infection, and treatment for GBS can effectively relieve headache symptoms.