Patients with intestinal failure have an increased risk for catheter-related bloodstream infections that can necessitate central venous line replacement and result in morbidity, prolonged hospitalization, or mortality. For pediatric patients with intestinal failure, the severe loss of intestinal absorptive ability leads to reliance on parenteral nutrition to meet minimal needs required for growth and development. Reliance on parenteral nutrition, in turn, forces dependency on central venous lines. Recent research concentrating on the pediatric population with intestinal failure indicates that prophylactic ethanol lock therapy can reduce the rate of catheter-related bloodstream infections and decrease central venous line removal rates in this high-risk population. Prevention of catheter-related bloodstream infections is critical for patients with intestinal failure. Ethanol lock therapy policies and protocols are increasingly being developed in healthcare institutions. Despite these efforts, no standard guidelines currently exist for ethanol lock therapy, and research in this area, specifically involving the pediatric population, is limited. This article presents the evidence to date as a means for assisting nursing professionals to make informed clinical decisions regarding the use of ethanol lock therapy for pediatric patients with intestinal failure.