Miller Fisher syndrome (MFS), a rare form of Guillain-Barré Syndrome, presents with the classical triad of ophthalmoplegia, areflexia, and ataxia. We describe the case of a 7-year-old boy who presented with diplopia, speech difficulty, dysphagia, gait disturbance, and difficulty in eyeball movements since 5 days. On examination, the child was having ataxia, areflexia, ophthalmoplegia, drooling of saliva, dysphonia, and absent gag reflex. MFS and brain stem encephalitis were kept as the differential diagnoses. The patient improved gradually over 3 weeks, following a treatment with intravenous immunoglobulin.