Malignant hyperthermia (MH) is a rare genetic myopathy that was first described as a fatal complication of general anesthesia in 1960. It is estimated to affect approximately 1 in 15,000 pediatric patients and 1 in 40,000 adult middle-aged patients. The mode of transmission is genetic: the severest form is autosomal dominant, and the less severe, autosomal recessive. Thus, both men and women can have MH, although there is a slightly higher incidence in the male pediatric population. Malignant hyperthermia is usually triggered by halogenated anesthetic agents with or without depolarizing muscle relaxants. The classic diagnostic triad consists of skeletal muscle rigidity, metabolic acidosis, and elevated body temperature. The definitive diagnosis is suspected susceptible individuals is revealed by exposing an intact muscle fiber to caffeine and halothane in varying concentrations. An abnormal contracture response is hypothesized to be the result of an increase in the release of calcium ion from the sarcoplasmic reticulum in response to neuronal stimulation leading to a hypermetabolic state. The mainstay of treatment is dantrolene, given either prophylactically in susceptible patients or immediately whenever a malignant hyperthermic episode is suspected.