Miller-Fisher syndrome is a peripheral neuropathy characterized by ophthalmoplegia, areflexia and ataxia. The incidence of this syndrome is estimated at 1 in a million, and just 14% of these occur on a pediatric age, making it an infrequent diagnosis in pediatrics. In this article we report a case of Miller-Fisher syndrome with an unusual onset on a pediatric patient along with infrequent radiological findings. (AU)