There is presented a clinical observation of such rare form of Hirschsprung’s disease in a newborn -as terminal aganglionosis of the ileum and hypo-ganglionosis of the cecum, manifested by acute intestinal obstruction. Resection of the ileocecal angle and leaving the distal colon in the child’s body with good functional results in the long-term postoperative period made it possible to affirm the existence of high isolated segmental forms of Hirshprung’s disease.