Patients with sickle cell disease (SCD) are prone to acute and chronic organ injuries that may necessitate surgical interventions earlier and with more frequency than non-SCD cohorts. They are also at an increased risk for perioperative morbidity and mortality because of the inherent pathophysiological derangements associated with SCD. Perioperative outcomes are influenced by phenotype variability, end-organ injury and the variable risks of surgical procedures, as well as the availability of beneficial therapies, especially where resources may be limited. Safe and effective perioperative management relies on anticipation and avoidance of complications, which can be best achieved by collaboration among anaesthesiologists, intensivists, surgeons and haematologists. This review addresses potential perioperative complications and contemporary management to assist in the perioperative care of paediatric patients with SCD.