Abstract | Decompressive craniectomy (DC)—a surgical procedure that involves removal of part of the skull to accommodate brain swelling—has been used for many years in the management of patients with brain oedema and/or intracranial hypertension, but its place in contemporary practice remains controversial. Results from a recent trial showed that early (neuroprotective) DC was not superior to medical management in patients with diffuse traumatic brain injury. An ongoing trial is investigating the clinical and cost effectiveness of secondary DC as a last-tier therapy for post-traumatic refractory intracranial hypertension. With regard to ischaemic stroke (malignant middle cerebral artery infarction), a recent Cochrane review concluded that DC improves survival compared with medical management, but that a higher proportion of DC survivors experience moderately severe or severe disability. Although many patients have a good outcome, the issue of DC-related disability raises important ethical issues. As DC and subsequent cranioplasty are associated with a number of complications, indiscriminate use of this surgery is not appropriate. Here, we review the evidence and present considerations regarding surgical technique, ethics and cost-effectiveness of DC. Prospective clinical trials and cohort studies are essential to enable optimization of patient care and outcomes.