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Miller Fisher syndrome, a variant of Guillain-Barre syndrome, is composed of the clinical triad of ophthalmoplegia, ataxia, and areflexia. A variety of other symptoms and signs have been reported in this syndrome, but headache is not a commonly reported symptom. We report a 35-year-old man with anti-GQ1b antibody-confirmed Miller Fisher syndrome presenting with severe and persistent headache, and we propose that the headache is caused by antibody-mediated effects on the trigeminovascular pain pathway.