Abstract: After regular anti-epileptic drug treatment, the symptoms of most patients with epilepsy can be well controlled or relieved, but 30%-40% of patients with epilepsy, after long-term drug treatment, still suffer from repeated seizures and develop refractory epilepsy. Lennox-Gastaut syndrome, Dravet syndrome and epilepsy with myoclonic-atonic seizures are all refractory epilepsy that originate in childhood and seriously threaten the physical and mental health of patients. In 2011, clobazam was approved by the US Food and Drug Administration for the adjunctive treatment of epileptic seizures in patients with Lennox-Gastaut syndrome aged≥2 years. The drug has also been used in the treatment of Dravet syndrome and epilepsy with myoclonic-atonic seizures. Currently, the mechanism of action of clobazam is still unclear, but it may exert pharmacological effects by binding to the benzodiazepine site on the γ-aminobutyric acid A receptor. In vivo, clobazam and N-desmethylclobazam are mainly metabolized by CYP3A4 and CYP2C19, and the interaction with other drugs should require vigilance in clinical application. Meanwhile, attention should also be paid to the blood concentration of N-desmethylclobazam and monitoring of drug-related adverse reactions in CYP2C19 poor metabolizers. To promote further standardization of clinical application of clobazam in our country, and to ensure the effectiveness and safety of clobazam, the Multi-disciplinary Team for Rare Diseases, Peking Union Medical College Hospital and the National Rare Diseases Committee organized experts and scholars in related fields, and after many discussions and revisions, finally formed this consensus for clinical reference.