Hirschsprung disease (HSCR) is a congenital disorder characterized by the absence of ganglion cells in the distal intestine, resulting in life-threatening functional bowel obstruction. While most children have disease limited to the rectosigmoid, the aganglionosis can extend further proximally, even affecting the entire intestinal tract in some cases. The condition affects 1 in 5,000 neonates and results from abnormal embryologic development of the enteric nervous system. Once the diagnosis is confirmed histologically by the absence of ganglion cells on rectal biopsy, surgical resection of the aganglionic segment is performed. Much variability exists in the operative approach and perioperative management of these patients, and outcomes are highly variable. Despite a seemingly successful operation, many children continue to experience constipation, enterocolitis, intestinal dysmotility, or varying degrees of fecal incontinence, and these can be due either to complications of the surgery or to functional defects inherent in the disease. Advancements in medical and surgical management, along with new insights into disease pathophysiology, are helping to improve outcomes for these children.