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Patients with febrile neutropenia (FN) may develop severe infection, septic shock, and death. To improve the outcome of pediatric oncology patients with suspected FN, clinical practice guidelines were developed for these patients at the emergency room (ER). The objective of the present study was to evaluate compliance of the clinical practice guidelines for children with cancer presenting with fever to the ER and adverse outcomes after using the guidelines.A retrospective cohort study was undertaken of children with cancer presenting with fever to the ER from January 2007 to December 2008 after the clinical guidelines were implemented. The control group was the children with cancer who presented with fever during January 2005–December 2006. Guideline compliance was evaluated by recording the time of initial clinical and laboratory assessment and door–to–antibiotic time. The adverse outcomes, including septic shock and death, were determined.There were 170 febrile episodes after using the guidelines. Approximately half (49.4%) of the patients received clinical assessment and laboratory results within 60 min, whereas the antibiotics were administered within 120 min in 80%. Prevalence of septic shock and intensive care unit admission were significantly reduced compared to controls (P = 0.011 and 0.016, respectively). No infection–associated mortality was found after the implementation of the guidelines.Using the clinical practice guidelines for pediatric oncology patients with fever was found to reduce the adverse outcomes and improve survival.