Guillain-Barre syndrome (GBS) is a common cause of acute flaccid paralysis in children. Axonal variants of this disease are rare, and frequently life-threatening or debilitating. The course and outcome of a 17-month-old child with acute flaccid paralysis including severe respiratory involvement are presented. GBS was suspected. Nerve conduction studies demonstrated acute motor-sensory axonal neuropathy including both phrenic nerves. The difficulties with the diagnosis and management of this severe and life-threatening condition are discussed. Significant morbidity is also highlighted. Axonal variants of GBS although rare cause significant morbidity in children. Diagnosis relies solely on accurate neurophysiologic testing and is important because the available treatment options for GBS are frequently ineffective in these variants.