Malignant hyperthermia (MH) is a life-threatening syndrome caused by sudden, uncontrolled skeletal muscle hypermetabolism in response to inhalation anaesthetics and depolarizing muscle relaxants. MH is related to an abrupt, massive release of calcium from the endoplasmic reticulum. False diagnosis and lack of targeted therapy are associated with high mortality rates; therefore, continuous education and knowledge updating are required. Our aim was to present the most recent data concerning epidemiology, genetics, pathophysiology, clinical course, treatment, and diagnosis of MH.