Malignant middle cerebral artery infarction is a life-threatening sub-type of ischemic stroke that may only be survived at the expense of permanent disability. Decompressive hemicraniectomy is an effective surgical therapy to reduce mortality and improve functional outcome without promoting most severe disability. Evidence derives from three European randomized controlled trials in patients up to 60 years. The recently finished DEcompressive Surgery for the Treatment of malignant INfarction of the middle cerebral arterY – II trial gives now high-level evidence for the effectiveness of decompressive hemicraniectomy in patients older than 60 years. Nevertheless, pressing issues persist that need to be answered in future clinical trials, e.g. the acceptable degree of disability in survivors of malignant middle cerebral artery infarction, the importance of aphasia, and the best timing for decompressive hemicraniectomy. This review provides an overview of the current diagnosis and treatment of malignant middle cerebral artery infarction with a focus on decompressive hemicraniectomy and outlines future perspectives.