Congenital megacolon is the third most prevalent congenital malformation in pediatric patients. It mainly presents with recurrent episodes of constipation and bloating. In the neonatal period, there is a delay in the expulsion of meconium, i.e., the meconium does not pass on its own within 24 hours after birth. The child requires manual assistance for defecation, after which recurrent abdominal distension and defecation dysfunction occur. This can often be relieved by a cathartic solution or enema. The diagnosis and treatment of congenital megacolon is complex. Typical children can be diagnosed by symptoms, physical examination and barium enema, while some atypical children require rectal anorectal manometry or even rectal mucosal biopsy. Once the diagnosis of congenital megacolon is confirmed, surgery is required. The purpose of surgery is to remove the entire diseased intestinal canal without ganglion cells. There are several surgical options, and the choice is individualized according to the condition. Since all procedures require transanal resection of the diseased intestinal canal, the protection of the anal bowel function is of utmost importance. The extent of bowel resection, the protection of bowel blood flow and the treatment of the myelin sheath all affect the postoperative recovery. We advocate laparoscopic-assisted radical megacolon resection for common and long-segment megacolon. Under direct intraperitoneal vision, the mesentery is released to the peritoneal reflex under the condition of protecting the colonic vascular supply, and then the diseased intestine is dragged out through the anus and resected and anastomosed. It has obvious advantages for the protection of anal function and intestinal blood flow.