A case of Miller Fisher and acute motor sensory axonal neuropathy (AMSAN) variant Guillain-Barré (MFS/AMSAN-GBS) overlap syndrome is presented. The neurological presentation of the overlap syndrome was preceded by an upper respiratory tract infection. Eventually, severe weakness of bulbar and limb muscles, areflexia, ophthalmoplegia, ataxia, and respiratory insufficiency developed. The electroneuromyography revealed symmetrical axonal polyneuropathy which was dominant in both upper limbs. Although a panel of anti-ganglioside antibodies including anti-GQ1b was negative, immediate treatment with intravenous immunoglobulin resulted in dramatic response.