A recent study and report in which hyperventilation was used during electroencephalography (EEG) in 6 children with sickle cell disease (SCD) and seizures, without serious complication, prompted a cautionary response regarding the potential risks attending the practice of EEG hyperventilation in SCD patients. Earlier reports of neurological impairment and stroke precipitated by the routine use of hyperventilation in children with SCD are reviewed, the mechanism and management of vascular infarction following hyperventilation are discussed, and readers are reminded of the AEEGS guidelines and contraindications to routine hyperventilation, which include SCD and trait and cerebrovascular disorders. The frequent nonobservance of these guideline recommendations among neurologists, and the need to more widely inform practitioners of the risks of hyperventilation in SCD are discussed.