To investigate the pathogenesis and clinical characteristics of rare clinical manifestations of seizures in patients with anti-GQ1b antibody syndrome. Methods: Clinical data of a case of anti-GQ1b antibody syndrome with seizures was reported and the literatures were reviewed. Results: The patient, a young female, presented with“over five months of unsteady gait and sudden onset of limb convulsions for one day”. The examination revealed symmetrical extraocular muscle paralysis, brisk tendon reflexes, and ataxia; serum anti-GQ1b IgG antibody was positive. The electroencephalogram showed bilateral hemispheric sporadic 3-4 Hz spikes and multiple spike discharges. Magnetic resonance imaging showed abnormal signals in the bilateral frontal lobes, left parietal lobe, bilateral thalamus, and brainstem. The patient was diagnosed with anti-GQ1b antibody syndrome, Bickerstaff brainstem encephalitis type. After treatment with antiepileptic drugs, steroids, and immunosuppressants, the patient improved. Four related case reports were collected, and basic medical science experiments suggested that GQ1b antigen was closely related to the pathogenesis of epilepsy. Conclusion: Seizure is a rare clinical manifestation of the anti-GQ1b antibody syndrome, which is speculated to be related to the distribution characteristics of GQ1b antigens, antibody concentration, and blood-brain barrier permeability. The underlying mechanisms still require further study.