Status epilepticus is a serious medical condition with annual incidence up to 120,000 cases in the United States alone, 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 article discusses the definition, clinical presentation and evaluation, and the management of generalized tonic-clonic status epilepticus. Literature review on epidemiology, etiology, pathophysiology, and treatment of acute status epilepticus and refractory status epilepticus are reviewed.