Congenital megacolon, also known as Hirschsprung’s disease, is caused by the congenital lack of intermuscular ganglion cells in a segment of the distal colon and the corresponding spastic contraction of the intestinal canal, resulting in functional stenosis and chronic dilatation and hypertrophy of the proximal colon causing various clinical symptoms. According to the length of the diseased intestinal tube, it can be divided into: short segment type, common type, long colon type and total colon type. The main clinical symptoms include: 1. delayed fecal excretion, more than 90% of children with megacolon have their first fecal excretion after 24 hours after birth; 2. chronic constipation, abdominal distension, growth retardation; 3. acute vomiting, abdominal distension, fever and other toxic symptoms in some children with combined necrotizing small intestinal colitis. Our department has made great progress in the basic and clinical research of congenital megacolon. Perfect preoperative examination and preparation, elaborate surgical design, and different surgical styles according to different types have greatly reduced postoperative complications. Total colon type megacolon and megacolon combined with small intestine colitis have high morbidity and mortality rate and disability rate. Our department has formulated strict treatment norms and accumulated rich experience in the diagnosis and treatment of total colonic megacolon and necrotizing small intestinal colitis, which has achieved satisfactory results and reached the domestic advanced level.