Background : Miller Fisher Syndrome usually presents with complete ophthalmoplegia, areflexia and ataxia. We present an unusual case which presented with unilateral abducens nerve palsy. Case presentation : A 51 year old female patient presented with a history of difficulty in walking and double vision for 1 week which started 2 weeks following an episode of acute gastroenteritis. She didn’t have any bladder bowel incontinence or difficulty in breathing. On examination there was left side abducens nerve palsy and bilateral significant dysmetria. Upper limb and lower limb power was 4/5 with global areflexia. There was no fatigability or sensory deficit. Higher cortical functions were intact with Glasgow Coma Scale of 15/15. Brain stem cerebro vascular accident, alcohol, toxin or drug mediated disease, myasthenia gravis, Bickerstaff encephalitis and Miller-Fisher syndrome were considered as possible differential diagnosis. There was no history of alcohol consumption or any exposure to drugs or toxins. Her Non contrast brain and MRI brain were normal. Nerve conduction study showed asymmetrical sensory motor and F wave abnormalities consistent with a Guillen-Barre Sydrome variant. Cerebro Spinal Fluid analysis showed albumino-cytological dissociation. These findings suggested the diagnosis of Miller-Fisher Syndrome. She was started on plasmaphareis. Her vital parameters, vital capacity and neurological deficit were closely monitored. With 5 cycles of plasmapharesis she made a complete neurological recovery and she was discharged on 16th day of admission. Conclusion: Miller Fisher Syndrome can present as unilateral abducens nerve palsy and early diagnosis and treatment leads to excellent functional outcome.