Congenital megacolon, also known as congenital anaplasia, is a relatively common developmental malformation of the gastrointestinal tract during the embryonic period, and is more common in males. Its pathogenesis is mainly related to genetic factors or changes in the microenvironment in the intestinal wall during embryonic development. The human intestinal system may seem to be just a soft tube, but the ganglia that govern its peristalsis are very complex. These ganglia begin to appear in the 5th week of embryonic development and by the 12th week can cover the wall of the digestive tract up to the rectum. When the intermuscular and submucosal neurons of the intestine are lacking or abnormally developed, this section of the intestine is in a state of continuous spastic contraction and loses its normal peristaltic function, thus making it impossible for the food residues to pass smoothly, and the normal intestinal canal at the upper end is dilated and hypertrophied due to the accumulation of feces, gradually forming a state of altered giant colon at the junction of stenosis and dilatation. This phenomenon can be clearly seen by barium enema. This phenomenon can be clearly seen by barium enema. Congenital megacolon usually presents with recurrent constipation after birth, due to the narrowing and obstruction of the intestinal tract of the child, resulting in obstruction of fecal discharge, which over time makes the child’s abdomen distended like a drum. However, some parents, especially in remote mountainous areas, do not know enough about congenital megacolon in children and do not take it seriously because they think their children are just having ordinary constipation, and simply treat it according to the treatment of constipation and leave it at that. Some parents, after learning that their child has congenital megacolon, think that the disease is impossible to cure or requires a lot of money for surgery, and often choose to give up treatment. If congenital megacolon is not treated surgically in time, it can lead to necrotizing small bowel inflammation, intestinal perforation, systemic electrolyte disorders, infectious shock, and multi-organ failure due to long-term severe stimulation, which threatens the life of the child. Since congenital megacolon is caused by the lack of intermuscular and submucosal neurons and abnormal development of the intestine, the muscles of the intestinal wall do not contract and cannot push the feces out of the intestine, resulting in defecation difficulties, the primary goal of treatment is to enable the child to defecate. At present, the radical treatment method to correct megacolon in China is surgery. The completely transanal modified Soave operation (scarless surgery) is performed by removing the abnormal bowel segment and the proximal dilated nerve developmental abnormal bowel segment through the natural lumen of the anal ganglion and dragging them out of the anus, and then anastomosing the normal intestinal canal with the anus. As for the timing of surgery, the earlier the diagnosis and surgical treatment, the less dilated the intestine will be, and the fewer intestinal segments will need to be surgically removed, which will also facilitate the recovery and reconstruction of intestinal function. Therefore, for children with a history of persistent constipation, parents should be vigilant and send them to a regular hospital for diagnosis as soon as abnormalities are detected.