The megacystis-microcolon-intestinal hypoperistalsis syndrome is part of a spectrum of intestinal motility disorders and is characterized by abdominal distension, lax abdominal musculature, incomplete intestinal rotation, microcolon, megacystis, bilious vomiting and decreased or absent intestinal peristalsis. In this report a newborn girl with megacystis-microcolon-intestinal hypoperistalsis syndrome is reported.