Abstract: Miller Fisher syndrome(MFS) is a likely variant of Guillain-Barre syndrome(GBS). Here, we present a case with peculiar demonstration of MFS as per the patient was found to have areflexia , ptosis left right distal with absent positional ,vibration sense and positive Romberg’s signs. There was history of pricking sensation in both Upper limb/Lower limb and swaying while walking, more towards the right. It was concluded as MFS from the clinical reports and the history taken. The infirm was started on methylprednisolone 1gm was given for 5 days followed by tapering dose of oral steroids along with Physiotherapy. Patient improved during the course in hospital and became stable. This event, point out the facts of a atypical disorder, which able us to limit the discrepancies to work out rapidly and properly handle such infirm. The anti GQ1b immunoglobulinG antibody is a definite marker of Miller Fisher syndrome, thus helps in the diagnosis of MFS. This moreover shows the importance of taking the history and medical assessment.