Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital disease with high mortality rate. The authors report a case of a female term infant with massive abdominal distension at birth. Abdominal ultrasonography revealed a huge cystic mass resolved after urinary catheterization. Exploratory laparotomy was performed and intraoperative findings were an enlarged urinary bladder, microcolon, short bowel and malrotation of the small intestine. Ladd’s procedure, ileostomy and vesicostomy were performed. Pathological findings of rectal biopsy revealed normal ganglion cells. Although prokinetic
drugs were given for promoting bowel motility, enteral feedings were not tolerated. She died from septicemia
at the age of 50 days.

Keywords: Megacystis, Microcolon, Hypoperistalsis, MMIHS