A 22-year-old female presented with a three day history of headache, unsteadiness and double vision. The headache was described as being occipital, constant, radiated to the front of the head, and was exacerbated by lying down. On direct questioning, it appeared that she had an acute diarrheal illness two weeks previously. Neurological examination was remarkable for a complete external ophthalmoplegia, areflexia and ataxia. A plain CT scan of the head was normal. A lumbar puncture revealed clear cerebrospinal fluid with an opening pressure of 29 cm H2O, a protein content of 0.42g/l, WCC of 0 and RBC of 82. Nerve conduction studies were normal. She was treated with a 5-day course of intravenous immunoglobulins and over the course of four weeks made a complete recovery but for the first week was greatly troubled by headache. The headache was not abolished by the lumbar puncture and became a considerable source of concern for the patient and family. The subsequent Anti GQ1b Antibodies were highly positive. Headache can be one of the presenting features of MFS but the aetiology is not clear. However it is important that clinicians are aware of headache as a possible feature of the condition, and that it appears to be self limiting.