Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) is a rare and the most severe form of functional intestinal obstruction in the newborn. The characteristic features of this congenital and fatal disease are abdominal distension, absent or decreased bowel peristalsis. Abdominal distension is a consequence of the distended, unobstructed urinary bladder with or without hydronephrosis. We present a case of female newborn with antenatal ultrasound revealing a large cystic mass in pelvic with urinary tract origin, abdominal distension, aperistalsis of the intestine and micro colon.