Abstract:  Objective To explore the features of nerve electrophysiology in Miller-Fisher syndrome (MFS) in children. Methods Thirty-four children with MFS were selected. The examinations of routine motor sensory nerve conduction, H reflexes of soleus muscle and F wave of median and tibial nerevs were performed. At the same time, 33 healthy children of  the same age were selected as control group and 30 children with typical Guillain-Barre syndrome (GBS) selected as GBS group, which were compared with children with MFS. Results In children with MFS, routine motor nerve conduction velocity and amplitude, and sensory nerve conduction velocity were in normal range. Twelve cases (35.29%) showed a decrease in sensory nerve amplitude potential (SNAP), 5 cases (14.71%) showed sural sparing phenomena (the decline degrees of SNAP in median or ulnar nerve greater than in sural nerve), 22 cases (64.71%) showed abnormality of H reflex, and 12 cases (35.29%) showed the decline of SNAP. Compared with control group, there was no difference in sensory nerve conduction velocity and SNAP in sural nerve in children with MFS (P<0.05); While there were significant decreases in SNAP in both median nerve and ulnar nerve (P<0.05). Compared with GBS group, children with MFS had significantly higher sensory conduction amplitude and faster velocity (P<0.01). Conclusion A decrease of terminal sensory nerve conduction amplitude could be found in children with MFS, but, the SNAP had limited impact on it. H reflex abnormality is the most common electrophysiologic abnormalities in MFS.