The true name of congenital megacolon is intestinal anaplasia, which is a congenital malformation of the development of the gastrointestinal tract due to the lack of ganglion cells in the lower distal part of the gastrointestinal tract and the persistent spasticity and narrowing of the diseased intestine, which prevents the expulsion of feces and results in dilated and hypertrophied proximal intestine. The main pathological change of congenital megacolon is the lack of ganglion cells in the distal part of the intestine. The cause of this disorder is multifactorial and may be due to impaired development of intestinal ganglion cells in early gestation due to viral infection or other environmental factors such as metabolic disorders and intoxication. The main etiologies identified and studied so far are early embryonic stage microenvironmental alterations and alterations in various genetic factors affecting the development of the enteric nerve. The incidence of familial congenital megacolon is about 5-20%, which is uncommon in Chinese, so the first baby with congenital megacolon will not necessarily occur in the second child. The chance of having a child with congenital megacolon is also not high. As long as children with congenital megacolon are diagnosed early and treated early, they generally do not affect nutritional absorption and growth, and even if growth and development are affected early, most children can still catch up after appropriate and active treatment. However, those children with particularly severe megacolon, such as total colon or total intestinal megacolon, will have their growth and development affected to some extent because there are too many diseased intestinal tubes and too few preserved intestinal tubes. Although the diagnosis and treatment of congenital megacolon has made great progress in recent years, there is still a certain mortality rate. The children who die are mainly those with severe small intestinal colitis complicated by megacolon, with megacolon crisis, systemic infection and poisoning; or severe types of megacolon leading to systemic metabolic disorders or chronic wasting death; and some children die due to severe perioperative infection, anastomotic dehiscence and hemorrhage.