Miller Fisher Syndrome (MFS) is an uncommon neurological disorder with an estimated yearly incidence of 1 in 1,000,000 in the United States. MFS is a variant of Guillain-Barre syndrome (GBS) characterized by ophthalmoplegia, ataxia, and areflexia, likely preceded by either a recent infection of the upper respiratory or gastrointestinal tracts, or vaccination. This syndrome most commonly affects adult males with a median age of 44 years. Here we want to draw attention to a rare case of MFS with atypical presentation in a 60-year-old female patient who presented to the Emergency Department (ED) with a medical history of hypertension and diabetes mellitus with complaints of blurred vision and walking difficulty for 2 days. She developed respiratory weakness requiring Intensive Care Unit (ICU) admission, and hyporeflexia, bilateral ptosis, and ophthalmoplegia subsequently. Provisional diagnosis of MFS was made upon cerebrospinal fluid (CSF) analysis which was later confirmed by the presence of Anti-GQ1b antibody. A single dose of Intravenous Immunoglobulin (IVIG) was sufficient to stabilize the patient’s condition, allowing her to be moved out of the ICU. Given the favorable prognosis of this disorder, early identification and effective drug treatment would minimize unnecessary medical interventions, curtail expenditures, and ease psychological distress.