INTRODUCTION: Gastrointestinal motility is produced by two types of smooths of muscles in the gastrointestinal tract which are circular muscles and longitudinal muscles. These muscles are stimulated by a complex multifaceted connection between intrinsic enteric nervous system and central nervous system. The enteric nervous system is composed of Interstitial cells of Cajal, submucosal plexus, myenteric plexus, migratory motor complex and gut neuromodulators. Gut motility disorders are derived from numerous etiologies that arise from the muscular, nervous, cellular, molecular components. Pediatric intestinal pseudo obstruction is a disorder that needs early diagnosis and prompt treatment to avoid associated life-threatening complications. CASE DESCRIPTION/METHODS: A 27 weeks gestational age baby girl born to a 25 year G1P0 mother with history of abruptio placenta and chorioamnionitis was admitted to the neonatal intensive care unit due to extreme low birth weight and prematurity. Patients developed disseminated Herpes Simplex Virus infection complicated with left grade 2 intraventricular hemorrhage. Afterwards, patient developed persistent total parenteral nutrition dependence, enteral food intolerance, marked intestinal dilation and constipation for 4 months since birth. Rectal biopsies rule out Hirschsprung disease. Multiple abdominal x-rays showed marked diffuse intestinal dilation. Water soluble enema study revealed inflammatory changes suggestive necrotizing enterocolitis. Patient was taken to the operation room since no clinical improvement. Intraoperative findings included viable intestinal tissue without ischemic change and no mechanical obstruction. After negative exploratory laparotomy we decided to perform a rigorous review of literature regarding intestinal dysmotility disorders. DISCUSSION: Our patient was diagnosed with pediatric intestinal pseudo obstruction based in our review of literature which is a intestinal motility disorder. Our plan was to perform multiple full thickness intestinal biopsies to confirmed diagnosis and surgical management with decompressive gastrostomy and ileostomy. Unfortunately, patient developed acute-clinical deterioration due to septic shock, catheter-related bacteremia and multiple organ failure. Functional motility disorders should be included as a differential diagnosis once mechanical obstruction rule out. Our aim is to provide awareness of early identification of PIPO and management since life-threatening complications may develop.