We report a 38-year-old woman who developed what appeared to be Fisher syndrome associated with optic nerve involvement. One week after a common cold, she developed double vision and left facial palsy. Four days after the onset, she developed bilateral blurred vision, painful total ophthalmoplegia, and ataxic gait. Brain CT and MRI findings were normal. Her vision worsened but the optic fundi were normal. Serum anti-GQ 1 b antibody was elevated. She received steroid therapy at another hospital, and her vision, facial palsy and ocular pain improved. She was transferred to our hospital and we treated her by plasma exchange. She showed near complete recovery. Human optic and ocular nerves contain high amount of GQ 1 b. This may be a underlying mechanism for optic nerve involvement in Fisher syndrome. We thought that she had an atypical Fisher syndrome associated with optic nerve involvement.