Status epilepticus is a serious medical condition with annual incidence up to 120,000 cases in the United States alone, (DeLorenzo et al 1996) of which more than 20% result in death. Generalized tonic-clonic status epilepticus is the most life-threatening form of status epilepticus. Increased awareness of this condition is essential in the management of patients with this condition. This review discusses the definition, the clinical presentation and workup, and the management of generalized tonic-clonic status epilepticus. Recent literature review on epidemiology, etiology, pathophysiology, and treatment of acute status epilepticus and refractory status epilepticus are reviewed.