Intestinal neuronal dysplasia is a common disease of chronic constipation in children, which is one of the Hirschsprung disease allied disorders. In recent years, the diagnostic methods of intestinal neuronal dysplasia have been improved, but diagnostic criteria are not standardized yet. The treatments include conservative treatment and surgical treatment. Special attention should be paid to save the integrity of the anal canal during operation. Moreover, using intestinal transplantation of neural stem cells and small intestinal transplantation to treat intestinal neuronal dysplasia has gained increasing attention.