OBJECTIVE: To investigate the clinical and electrophysiological characteristics and prognosis of acute motor axonal neuropathy (AMAN) in children in South China. METHODS: The clinical and electrophysiological data of 6 children with AMAN was analyzed, and they were followed up. RESULTS: The mean age of onset was 4.4 years. Most patients came from rural areas and 5 cases had a history of prodromal infection. There were no seasonal differences in clinical onset among the patients. The most common first symptom was muscle weakness, and the mean time from onset to the most severe disease status was 4.2 days. Nerve conduction test results revealed that all patients showed significantly lower amplitude of motor nerve action potential, only 22.3%-73.4% of the lower limit of normal. Injury to the nerves of distal extremities was more serious than injury to the nerves of proximal extremities (P<0.05), while there was no significant difference in the injury to the nerves of upper and lower extremities (P>0.05). Motor nerve conduction velocity and sensory nerve conduction velocity were normal. All patients received intravenous immunoglobulin (IVIG). Of the 6 AMAN patients, 4 could walk independently after a follow-up of 3 months to 1 year. CONCLUSIONS: AMAN in children occurs mostly in rural areas. There is no seasonal difference in the clinical onset of the disease. Muscle weakness is the most common first symptom and the worst status of AMAN appears in the early stage of the disease. Electrophysiological examination provides important information for the diagnosis of AMAN. Some children with AMAN regain the ability to walk independently 1 year after onset. Early application of IVIG treatment may help recovery of neural function.