The authors present the cases of three patients with total aganglionosis of the colon and review the literature. The main diagnostic features are reviewed to justify a separate analysis of the typical form of Hirschsprung's disease. There are no pathognomonic radiological findings and definitive diagnosis can only be established by histological examination. Considering the high incidence of complications and the mortality of patients with total aganglionosis of the colon, this disease should be suspected in any infant or young child with evidence of bowel obstruction seen on plain films, regardless of the contrast enema findings.

Keywords: Aganglionosis, Hirschsprung's disease, Intestinal obstruction