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Miller Fisher syndrome (MFS) is an uncommon variant of Guillain-Barré syndrome (GBS). It is characterized by external ophthalmoplegia, ataxia, and areflexia. The incidence of MFS as a proportion of GBS was reported to be 1 to 5 % in western countries and considerably higher in Eastern Asia i.e. 19% in Taiwan and 25% in Japan. Here we report the clinical features of a patient who initially presented with ataxia then subsequently developed limb weakness, areflexia and ophthalmoplegia. CSF study and NCV study supported our clinical diagnosis. Patient was managed conservatively. No intravenous immunoglobulin was given but the patient gradually improved in symptoms including power, ataxia, ophthalmoparesis. After one month the patient was discharged from hospital with complete recovery. DOI: http://dx.doi.org/10.3329/bsmmuj.v5i1.11028 BSMMU J 2012; 5(1):69-71