Acute ophthalmoparesis without ataxia (AO) is an atypical form of MillerFisher syndrome (MFS) and is rare in children. Anti-GQ1b antibodies canbe detected in patients with AO, as in MFS. A 5.5-year-old girl had totalophthalmoparesis, blurred vision, ptosis, diplopia and mydriasis non-reactiveto light or near stimuli with preserved consciousness and deep tendon reflexes.She had no ataxia. Cerebrospinal fluid (CSF) examination and cranial MRIwere normal. Serum antiGQ1b antibodies were positive. She was diagnosedwith AO and intravenous Immunoglobulin (IVIG) was ordered, 400 mg/kg/day, for 5 days. Ophthalmoparesis and blurred vision improved in a fewweeks. At the end of the first year, mydriasis still persisted, but improvedand became responsive to near stimuli. Pupillary involvement may be seenin approximately 50% of MFS patients, and improvement in a few weeks ormonths has been reported in adults. Our case shows the expanding clinicalspectrum of anti-GQ1b positive cranial neuropathy as early-onset AO andprolonged mydriasis more than one year.