Background/Purpose: Abnormality of innervation of externa I anal sphincter is one of the most important factors affecting postoperative anorectal function. The abnormalities of lumbosacral nerves have been reported in many radiological and histopathologic studies. There are few reports on the neurophysiological changes in children with anorectal malformation. The aim of this study was to examine the functional changes of nerves to the external anal sphincter in anorectal malformation. Methods: Forty-five patients with anorectal malformation underwent studies of latencies of pudendo-anal reflex, spinoanal response, and evoked potential of cauda equine simultaneously. The conduction time of afferent nerve, efferent nerve, and sacral spinal center of pudendo-anal reflex are were analyzed quantitatively. Results: The latencies of pudendo-anal reflex, spine-anal response, and conduction time of sacral spinal center significantly are prolonged in patients with anorectal malformation (P < .05). There was significant difference between rectourethral fistula group and vestibular fistula group as well as low-type deformity group. The patients with lumbosacral anomalies such as lumbosacral spinal bifida or absence of S4 or S5 had prolonged latencies, especially the pudendo-anal reflex latency. There was significant negative correlation between the latencies and clinical scores. Although pudendoanal reflex latency was longer in patients who had posterior sagittal anorectoplasty than those who had abdominoperineal pull-through procedure, the difference was not significant. Conclusions: The abnormality of nerves to external anal sphincters is one of the important causes for clinical outcome. The neural lesions vary in each type of anal and lumbosacral deformity. Copyright (C) 2000 by W.B. Saunders Company.