The case of a newborn girl with the megacystis-microcolon-intestinal hypoperistalsis syndrome is described. The patient had a huge bladder without distal obstruction. Although a laparotomy revealed malrotation and a microcolon, the main cause of the intestinal obstruction was hypoperistalsis. Intestinal hypoperistalsis persisted without any improvement and hyperalimentation was required. The outcome was fatal as in most of the reported cases.

Keywords: bladder, colon, intestinal obstruction, megacystis-microcolon-intestinal hypoperistalsis syndrome, newborn