A case of intestinal pseudo-obstruction in the neonatal period is presented. It is known as Megacistis-microcolon-intestinal Hypoperistalsis Syndrome and is a rare cause of intestinal dismotility. A white female newborn weighing 3,110 g was admitted to the Neonatal Surgical Unit of the Hospital de Base with a small omphalocele and a history of biliary vomit. Abdominal X-rays revealed complete absence of intestinal gas. Exploratory laparotomy was carried out, and the findings were great distension of the bladder, a short poorly developed and malrotated bowel with a non-fixed cecum. Surgical treatment consisted of gastrostomy, sigmoid colostomy, vesicostomy and repair of the omphalocele. Multiple biopsies were done in the small and large bowel. Ganglion cells were present in all specimens. The patient did not tolerate enteral feeding, and total parenteral nutrition was readily initiated. Nonetheless, death occurred on the 51st postoperative day. This is a rare syndrome which presents itself at birth as chronic intestinal obstruction. A postmortem examination confirmed the diagnosis.