Background: Status epilepticus (SE) is a major medical emergency and requires not only an emergency symptomatic treatment with antiepileptic drugs (AED) but also a rapid identification and treatment of the underlying cause. This narrative review summarizes the most important advances in SE classification and treatment. Data sources included being PubMed / Medline, and tracking references of the relevant studies, reviews and books. SE is now defined as “a condition resulting either from the failure of the mechanisms responsible for seizure termination or from the initiation of mechanisms, which lead to abnormally prolonged seizures (after time point t1). It is a condition, which can have long-term consequences (after time point t2), including neuronal death, neuronal injury, and alteration of neuronal networks, depending on the type and duration of seizures.” The most effective acute treatments for early SE are the intravenous benzodiazepines (lorazepam, diazepam, and clonazepam) and intramuscular midazolam. In children, oral or intranasal midazolam are useful alternatives. The intravenous antiepileptic drugs (phenytoin, valproate, levetiracetam, phenobarbital and lacosamide) are administered in confirmed SE. Treatment options in refractory SE are intravenous anesthetics; ketamine, magnesium, steroids and other drugs are used in super-refractory SE , showing variable results and outcomes. Conclusions: Over time, major progress has been made in defining, classifying, and understanding of SE mechanisms. Despite this, the first-line drug management is ineffective in up to 40% of patients with SE. The super-refractory SE treatment is still unknown and no evidence-based data have been found yet. Thus, SE treatment strategies vary substantially from one institution to another due to the lack of data supporting a specific treatment plan.