Fever in the pediatric population is a common chief complaint presenting to the emergency department and may be one of the first indications of a life-threatening infection, especially in patients with neutropenia. Given that pediatric patients with febrile neutropenia frequently present to emergency departments for emergent care, it is critical for emergency medicine physicians and pediatricians and family physicians working in the emergency department to know the key aspects of the clinical approach to these patients. This review of the clinical evaluation and treatment of the pediatric patient presenting with fever and confirmed or suspected neutropenia will provide health care providers with the necessary tools to effectively care for this patient population.