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Exertional heat illness and malignant hyperthermia (MH) are acute and life‐threatening disorders that share comparable clinical features, such as tachycardia, rhabdomyolysis and hyperthermia. Therefore, in several case reports, a possible link between both has been discussed. However, the number of these reports is small and systematic investigations nearly impossible. This review presents all relevant aspects of MH and exertional heat illness. Furthermore, the author discusses the association between both disorders. The main conclusions are as follows: (1) only a minority of patients with an exertional heat illness have an underlying skeletal muscle defect; (2) most patients with MH should not modify their physical activity; and (3) only those patients with exertional heat stroke not responding to symptomatic therapy should be treated with dantrolene. These conclusions can be supported completely; however, it would be desirable to develop guidelines for investigating patients with exertional heat illness, especially with respect to MH susceptibility.