This report describes 51 neonates with meconium ileus and emphasizes a changing pattern of treatment and improved survival. Twenty-four neonates had uncomplicated meconium ileus due to inspissated meconium obstructing the distal ileum. Twenty-seven neonates had 41 complications of meconium ileus including volvulus (18), bowel atresia (13), perforation (5), and giant cystic meconium peritonitis (5). Nine patients with uncomplicated cases responded to nonoperative clearing of meconium using a meglumine diatrizoate (Gastrografin) enema. Six of 7 patients with enema failures underwent laparotomy, purse-string enterotomy, and intraluminal irrigation. The remaining 9 patients with uncomplicated meconium ileus had resection and enterostomy. Complicated cases were managed by resection and anastomosis (13) or enterostomy (14). Survival at 1 year was 92% in patients with uncomplicated meconium ileus and 85% for those with complicated meconium ileus. Nonoperative Gastrografin enema or enterotomy-irrigation can relieve obstruction in uncomplicated meconium ileus and avoid an enterostomy in most cases.