Introduction: Miller Fisher is a known variant of Guillain Barre Syndrome with the triad of ophthalmoplegia, areflexia and ataxia. This case will discuss a rare presentation with a severe headache. Case Presentation: A 51 year old male initially presented with blurry vision and a severe occipital headache with no papilledema. Patient endorsed having an upper respiratory-like illness 1-2 weeks prior to symptoms onset. Initial head CT was negative. The lumbar puncture was performed, and noted to have an opening pressure of 320 mmHg. Other values were normal. Serum studies were positive for GQ1B antibody. This gave the diagnosis of Miller Fisher Syndrome. IVIG treatment was initiated and symptoms largely improved, including the headache. Severe headache is usually not a symptom of Miller Fisher Syndrome. In a case series including 27 patients with Miller Fisher Syndrome, only two reported a headache. One study proposed that an increase in CSF protein could lead to outflow obstruction, increasing ICP and leading to headaches. However our patient’s CSF protein was 45 mg/dl. Another potential explanation could be injury to the ventral and dorsal roots of cranial nerves by antibodies to GD3 and GD1b. A different study discovered that along with antibodies to GQ1b, in rare cases patients also develop antibodies to GD3 and GD1b. With such a small percentage of patients being positive for the antibodies to GD3 and GD1b, it was theorized that this could be the reason for the headache. Unfortunately, in our case, both GD1b and GD3 antibodies were negative. Discussion: Headaches are a rare symptom of Miller Fisher with unknown pathophysiology. More research is needed, especially in antibody related nerve damage. Nonetheless, physicians need to identify this symptom and know headaches are usually self-limiting.