Extensive aganglionosis very close to the ligament of Treitz and total intestinal aganglionosis are rare forms of Hirschsprung's disease. In these cases, nutrition, fluid, and electrolyte balance are a problem. Although the myectomy-myotomy technique of Ziegler appears hopeful, no effective surgical method has been put into the practice for the treatment of these rare forms of Hirschsprung's disease. We report four cases with extensive intestinal aganglionosis, one of which was total intestinal aganglionosis with involvement of the stomach.