© Springer International Publishing Switzerland 2016. Most pediatric patients with irreversible intestinal failure are dependent on parenteral nutrition (PN) and eventually develop life-threatening complications from long-term PN. Intestinal transplantation is now an accepted treatment for the pediatric population developing irreversible intestinal failure resulting from either short gut syndrome or functional bowel problems with preserved length. Over the years several modifications in immunosuppressive strategies and refinements in the surgical techniques have helped lower the mortality and improve the outcomes of intestinal transplantation. Despite this, we continue to face the unresolved challenge of graft loss from chronic rejection and significant morbidity from infectious complications and drug toxicities with aggressive immunosuppression. However, positive weight gain and growth reported in pediatric recipients along with freedom from PN indicate successful gastrointestinal autonomy after intestinal transplantation. A preserved quality of life, with cognitive, psychosocial, and physical functions comparable to healthy normal children, makes intestinal transplantation a reasonable option for irreversible intestinal failure.