Miller Fisher syndrome is characterized by a triad of ataxia, ophthalmoplegia, and reduced or absent tendon reflexes, with minimal if any limb weakness. Anti-GQ1b immunoglobulin G antibodies are present in high titers in most patients. Presented is a two-year-old female with Miller Fisher syndrome whose blood serum was negative for anti-GQ1b immunoglobulin G antibodies. (C) 2003 by Elsevier Inc. All rights reserved.