IntroductionIntestinal failure, defined as the loss of gastrointestinal function to the point where nutrition cannot be maintained by enteral intake alone, presents numerous challenges in children, not least the timing of consideration of intestine transplantation.ObjectivesTo describe the evolution of care of infants and children with intestinal failure including parenteral nutrition, intestine transplantation, and contemporary intestinal failure care.MethodsThe review is based on the authors' experience supported by an in‐depth review of the published literature.ResultsThe history of parenteral nutrition, including out‐patient (home) administration, and intestine transplantation are reviewed along with the complications of intestinal failure that may become indications for consideration of intestine transplantation. Current management strategies for children with intestinal failure are discussed along with changes in need for intestine transplantation, recognizing the difficulty in generalizing recommendations due to the high level of heterogeneity of intestinal pathology and residual bowel anatomy and function.DiscussionAdvances in the medical and surgical care of children with intestinal failure have resulted in improved transplant‐free survival and a significant fall in demand for transplantation. Despite these improvements a number of children continue to fail rehabilitative care and require intestine transplantation as life‐saving therapy or when the burden on ongoing parenteral nutrition becomes too great to bear.