The aim of this study was to investigate the clinical electrophysiological features and prognosis of Acute Motor Axonal Neuropathy (AMAN) in Uygur children of Xinjiang province. Six patients, aged 2 to 12 years, were included in the study. Five of the six patients had a history of prodromal infection. Disease onset occurred between June and December, with myasthenia as the main onset sign (100%). The mean peak time of the disease was 5.8 d. The amplitude of motor nerve potential in all children decreased (21.1-70.3% of the normal lower limit), the degree of nerve injury at the distal end was greater than at the proximal end (P<0.05), and the degree of injury of the upper and lower extremity nerves showed no significant difference (P>0.05). The motor and sensory nerve conduction speeds were normal, and gglobulin was present. Myasthenia is the most common onset sign of AMAN in Uygur children, though multiple disease symptoms can reach their peak in early stages of the disease. Electromyography (EMG) is of great significance in the diagnosis and differential diagnosis of AMAN. Early Intravenous Immunoglobulin (IVIG) therapy is efficacious in promoting the recovery of clinical neurological function.