<p>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, typically following a recent upper respiratory or gastrointestinal infection, or less commonly, vaccination. While MFS most often affects adult males, with a median age of 44, we highlight a rare, atypical case in a 60-year-old female patient who presented to the Emergency Department (ED) with complaints of blurred vision and walking difficulty for 2 days. Her condition rapidly worsened, leading to respiratory weakness requiring Intensive Care Unit (ICU) admission, with hyporeflexia, bilateral ptosis, and ophthalmoplegia subsequently developing. MFS was provisionally diagnosed via cerebrospinal fluid (CSF) analysis and later confirmed by the presence of anti-GQ1b antibodies. Remarkably, a single dose of Intravenous Immunoglobulin (IVIG) stabilized her condition, allowing transfer out of the ICU and gradual recovery. Although ophthalmoplegia is the most prevalent finding in MFS, it may not be present initially. Besides, in some cases, areflexia may not be present to fully complete the clinical triad. Ataxia, the hallmark of cerebellar stroke, is the initial sign in a significant minority of patients. Our case demonstrated all of these atypical features simultaneously, making it a valuable clinical example. Recognizing such presentations of MFS that mimic acute stroke reinforces the need to consider MFS as an early differential for sudden-onset ataxia, thereby preventing unnecessary medical interventions, lower healthcare costs, and promote early rehabilitation.</p>