Objective To investigate the clinical, electrophysiological and prognosis of patients with acute anti-GQ1b antibody syndrome. Methods A retrospective analysis was performed on the clinical data of 24 children diagnosed with acute anti-GQ1b antibody syndrome between 2019 and 2023 at our hospital. Demographic characteristics, clinical manifestations, electrophysiological findings, and outcomes were systematically reviewed and analyzed. Results All 24 patients presented with acute onset of symptoms. The median age was 4.0 ( 2.0-13.5) years, with male predominance (62.5%). Common clinical features included reduced or absent tendon reflexes, ataxia, limb weakness, sensory disturbances, and cranial nerve involvement. Autonomic dysfunction was observed in 4 patients, and lethargy was noted in 2.Nine patients exhibited clinical features consistent with the Miller-Fisher syndrome (MFS) spectrum, including 5 with classical MFS and 4 with variant MFS. Eight patients displayed Guillain-Barré syndrome (GBS)-related phenotypes, comprising 3 classical GBS and 5 variant GBS cases. The remaining 7 patients were classified as mixed type, presenting overlapping features of MFS, GBS, and Bickerstaff’s brainstem encephalitis (BBE). In addition to anti-GQ1b antibodies, 15 patients had detectable levels of other anti-ganglioside antibodies, most commonly anti-GT1a and anti-GM1. Cerebrospinal fluid (CSF) analysis revealed protein-cell dissociation in 21 patients. Electrophysiological studies showed mild sensory nerve amplitude reduction in the MFS group, whereas both motor and sensory nerves were significantly affected in the GBS and mixed-type groups. All patients received intravenous immunoglobulin therapy. After 3-month follow-up, 15 patients demonstrated favorable neurological recovery. Patients with poor recovery were more likely to have cranial nerve injury, autonomic dysfunction, and higher GDS scores upon admission (P<0.05). Conclusion Acute anti-GQ1b antibody syndrome in children demonstrates a wide range of clinical phenotypes, with both axonal and demyelinating pathologies present. Follow-up nerve conduction studies conducted between 3 and 8 weeks post-onset are valuable for assessing electrophysiological subtypes and monitoring recovery. Approximately two-thirds of affected children achieve good functional outcomes, while those with cranial nerve involvement, autonomic dysfunction, or elevated initial GDS scores tend to have poorer prognosis.