A 15 year old patient developed ataxia 2 weeks after an upper respiratory infection. Absent reflexes, external ophthalmoplegia, bilateral ptosis, isochoric mydriasis and week photomotor reflexes (Bell's phenomenon) were noted. A slight increase in protein but not cell content of the CSF was observed. Miller-Fisher syndrome was diagnosed on clinical grounds and visual and auditory evoked potentials were explored and found to be normal. This is in complete agreement to the literature and indicates indemnity of the central nervous system in Miller-Fisher's syndrome. Peripheral nervous system abnormalities that have been identified by pathologic and radiologic studies may explain all of the alterations observed in this syndrome.