We review the types and causes of convulsive status epilepticus (CSE) in infants and young children in Japan, and discuss the current recommendations for the use of intravenous (IV) drugs in managing this condition, and report on our clinical experiences. There are prolonged or continuous CSE and clustered or intermittent CSE, and treatments are different between them. In Japan, fosphenytoin and IV preparation of lorazepam and phenobarbital are not available. Recently, midazolam and lidocaine (LDC) have been widely used, although neither of these drugs have official approval for the management of CSE. Febrile seizures and epilepsies are common causes of CSE in infants and young children in Japan, followed by benign infantile convulsions (BIC), convulsions with gastroenteritis (CwG), and acute encephalitis with refractory CSE and intractable epilepsy (AECSEE), which are familiar disorders in Japan. BIC and CwG frequently present with clustered CSE and do not respond to IV diazepam, but have an excellent response with oral carbamazepine or IV LDC. CSE in AECSEE requires control with barbiturate coma. The Research Committee on Clinical Evidence of Medical Treatment for Status Epilepticus in Childhood has developed a proposed guideline for the treatment of CSE in childhood in Japan by an evidence-based approach and consensus conference. Initial management of seizures should be attempted mainly with IV diazepam, the second-line treatment involves IV midazolam followed by IV phenytoin if seizures persist, and the third-line treatment requires barbiturate coma. However, our experience of 247 episodes of CSE in 77 patients, predominantly with chronic epilepsy, required different second-line treatments for prolonged CSE compared with clustered CSE: the former were treated with IV midazolam or pentobarbital, and the latter were given IV phenytoin or LDC. We propose modifications to the guideline for CSE that the second-line treatment is divided by prolonged CSE and clustered CSE, and that the procedures for brain protection and systemic management are added.