To analyze the clinical features and prognosis of Miller-Fisher Syndrome Manifesting (MFS) with isolated ophthalmoplegia. Methods: We report two cases of MFS presenting with bilateral isolated ophthalmoplegia as the primary clinical manifestation followed by a systematic review of relevant literature. Results: Case 1 involved a 54-year-old female presenting with diplopia and bilateral blepharospasm following infection. Case 2 described a 67-year-old male who developed diplopia without clear triggers, accompanied by unilateral ptosis, dilated pupils with sluggish light reflex, and ocular motility disorders. Both patients tested positive for serum anti-GQ1b IgG antibodies and anti-GT1a IgG antibodies; Intravenous immunoglobulin therapy was administered to both patients, leading to favorable outcomes. By integrating our two new cases with previously reported instances, we identified a total of seven patients manifesting isolated ocular symptoms: five were anti-GQ1b IgG positive, six exhibited external ophthalmoplegia, five had internal ophthalmoplegia, five reported prodromal infections, and all achieved good recovery. Conclusion: Patients with MFS presenting solely with ocular symptoms are exceedingly rare. These cases significantly enhance our understanding of this distinctive clinical entity.