Objective: to investigate the characteristics and clinical value of evoked potentials in late infantile form of metachromatic leukodystrophy. Methods: Brainstem auditory, and somatosensory evoked potentials were recorded in 6 patients, and compared with the results of CT scan. Results: All of the 6 patients had abnormal results of BAEP and MNSEP. The main abnormal parameters in BAEP were latency prolongation in wave I, inter-peak latency prolongation in Ⅰ-Ⅲ and Ⅰ-Ⅴ. The abnormal features of MNSEP were low amplitude and absence of wave N9, inter-Peak latency prolongation in Ng-N13 and N13-N20, but no significant change of N20 amplitude. The results also revealed that abnormal changes in BAEP and MNSEP were earlier than that in CT. Conclusion: The detection of BAEP and MNSEP in late infantile form of metachromatic leukodystrophy might early reveal the abnormality of conductive function in nervous system and might be a useful method in diagnosis.