Guillain-Barre syndrome (GBS) is a serious neurological disorder associated with a rapid progressive ascending muscle paralysis, and it is the most common neurological autoimmune disorder that affects the peripheral nervous system, which is usually triggered by viral or bacterial infection. GBS is rare in children and characterized by rapid progressive onset ascending muscle weakness associated with pain and sensory dysfunction. Miller Fisher syndrome (MFS), a variant of GBS, is rare in pediatric population which is typically manifested by ataxic gait, ophthalmoplegia, and areflexia since it is rare in children. It is vitally important to early diagnose this condition and to initiate early treatment to prevent further complications and long-term morbidity. Since the outbreak of Zika virus, the incidence of GBS has been increased. Zika virus associated with autoimmune anti-ganglioside antibodies trigger which lead to GBS development. Zika virus infection should be strongly considered in patients who present with classical signs of Miller Fisher syndrome, especially travelers and residents from endemic areas.