Approximately 5000 children present to the emergency department annually with unintentional carbon monoxide poisoning. Children may be more vulnerable to carbon monoxide poisoning because of their increased metabolic demand and their inability to vocalize symptoms or recognize a dangerous exposure, and newborn infants are more vulnerable to carbon monoxide poisoning because of the persistence of fetal hemoglobin. Mild carbon monoxide poisoning may present as viral symptoms in the absence of fever. While headache, nausea, and vomiting are the most common presenting symptoms in children, the most common symptom in infants is consciousness disturbance. This review discusses the limitations of routine pulse oximetry and carboxyhemoglobin measurement in determining carbon monoxide exposure, and notes effects of co-ingestions and comorbidities. Although the mainstay of treatment is 100% oxygen, the current evidence and controversies in the use of hyperbaric oxygen therapy in pediatric patients is reviewed, along with its possible benefit in preventing delayed neurologic sequelae.