Collier's sign is well known as unilateral or bilateral eyelid retraction due to midbrain lesions. This sign is usually caused by infarction, tumor, multiple sclerosis, neuro-degenerative disease, or encephalitis. We report a case of Miller Fisher syndrome (MFS) which demonstrated Collier's sign. A 54-year-old man developed ophthalmoplegia, ataxia, and areflexia two weeks after common cold-like symptoms. At the same time, bilateral upper eyelid retraction (Collier's sign) was remarkably observed. Serum anti-GQ1b antibody was positive. Albumino-cytologic dissociation was seen at two weeks after onset. We treated him with high dose intravenous immunogloblins (IVIg) for five days. There was remarkable improvement after the administration of IVIg, and there was a complete recovery from his eyelid retraction. All his symptoms of MFS also disappeared. The eyelid retraction of Collier's sign has been reported to occur with lesions in the rostral midbrain and posterior commissure. Therefore, Collier's sign in this patient suggested central nervous system involvement in the MFS. To our knowledge, this is the first report of MFS associated with Collier's sign.