Background: The Miller-Fisher variant of Guillain-Barré syndrome (MFS-GBS) is a rare subtype of the autoimmune neuropathic disorder that is characterized by a unique clinical triad of ophthalmoplegia, ataxia, and areflexia. While the peripheral nervous system is primarily affected, there is growing evidence of autonomic nervous system (ANS) involvement in MFS-GBS. Objective: This review aims to examine the frequency of ANS involvement in patients with MFS-GBS, exploring its clinical manifestations, underlying pathophysiology, and implications for diagnosis and management. Methods: A comprehensive literature search was performed in electronic databases, including PubMed, Embase, and Google Scholar, using the keywords "Miller-Fisher variant," "Guillain-Barré syndrome," “autonomic dysfunction” “dysautonomia” and "autonomic nervous system." Articles published in English from inception to September 2022 were considered. Studies reporting on the frequency and clinical manifestations of ANS involvement in MFS-GBS were included. Relevant studies were identified and included in this review. Result: The findings suggest that ANS dysfunction is uncommon in MFS-GBS, with various autonomic symptoms reported, including cardiovascular, gastrointestinal, and sudomotor dysfunctions. Early recognition and appropriate management of ANS involvement are essential for optimizing patient outcomes. Conclusion: Further research is needed to better understand the mechanisms underlying ANS dysfunction in MFS-GBS and to develop targeted therapeutic interventions. Keywords: Miller-Fisher variant, Guillain-Barré syndrome, autonomic nervous system, cardiovascular dysfunction, gastrointestinal dysfunction, sudomotor dysfunction