Objectives To compare clinical manifestations and electrophysiological features in patients with chron?ic inflammatory demyelinating polyneuropathy (CIDP) and Type-I Charcot Marie Tooth Disease (CMT-I) for guiding dif?ferential diagnosis. Methods Data including clinical manifestations and electrophysiological indexes was collected from thirty-one CIDP cases and 28 CMT-I cases. Correlation analysis was used to assess the association of the severity of electrophysiology with the severity of clinical symptoms. Results There were statistically significant differences in onset site, sensory dysfunction, foot deformity and cerebrospinal fluid protein between these two groups (P<0.05). There were significant differences in indexes of nerve conduction and needle electromyography between these two groups (P<0.05). The severity of clinical symptoms was not related with the severity of electrophysiology in CMT-I group (r=0.27,P>0.05). Conclusions Differential diagnoses of CIDP and CMT-I can be made based on clinical manifestations and electro?physiological features.