A 2-month-old girl with acute lead poisoning demonstrated electrophysiologic evidence of neurotoxicity. Motor nerve conduction studies of the median, ulnar, peroneal, and posterior tibial nerves revealed both axonal and demyelinating neuropathy. Somatosensory evoked potential studies of median and posterior tibial nerves demonstrated evidence of cortical involvement. Brainstem auditory evoked potential study disclosed the possibility of acoustic nerve involvement but no evidence of a brainstem lesion. Postmortem examination revealed cerebral edema and focal segmental demyelination of the median nerve.