Malignant catatonia (MC) represents a life-threatening neuropsychiatric disorder that was widely reported long before the introduction of antipsychotic drugs. A review of the world literature on MC indicates that although the prevalence of the condition may have declined since the pre-antipsychotic drug era, it continues to occur and represents a syndrome rather than a specific disease. Although most often the outgrowth of a psychiatric disorder, MC may develop in association with diverse neurologic and medical conditions. From this perspective, neuroleptic malignant syndrome (NMS) may be viewed as a drug-related form of this same MC syndrome. Our review also supports the proposed conceptualization of catatonia as a continuum, with milder forms at one end (termed simple or nonmalignant catatonia) and more severe forms involving hyperthermia and autonomic dysfunction (termed malignant catatonia) at the other end. In addition, findings from our review suggest that simple catatonia, MC and NMS share a common pathophysiology involving reduced dopaminergic functioning in the basal ganglia-thalamocortical circuits, consistent with their identity as variants of the larger unitary catatonic syndrome. Electroconvulsive therapy appears to represent the preferred treatment for MC. Antipsychotic drugs should be withheld whenever MC is suspected.