The diagnosis of vitamin B6-dependent epilepsy relies on clinical symptoms, mainly response to treatment with pyridoxine and relapse off medication, while relevant genetic testing may be performed to confirm the diagnosis. Vitamin B6-associated epilepsy is a group of epilepsy-related disorders in which seizures cannot be controlled by antiepileptic drugs but can be controlled or significantly improved by vitamin B6, mainly including pyridoxine-dependent epilepsy and pyridoxine-responsive epilepsy. Pyridoxine-responsive epilepsy latter also includes vitamin B6-responsive infantile spasms, pyridoxal (amine) phosphate oxidase (PNPO) deficiency, and hyperphosphatasemia with mental retardation syndrome (i.e., Mabry syndrome). After six weeks of successful control of convulsions, vitamin B6-dependent epilepsy reoccurred. Also, seizures recur after discontinuation of vitamin B6, and vitamin B6 supplementation within the first eight months of onset does not control seizures, but can be successfully controlled thereafter. Confirmation of the diagnosis of vitamin B6-dependent epilepsy relies on genetic testing. a – AASA and PA testing can assist in the diagnosis, and mutation analysis of the ALDH7Al gene can confirm the diagnosis. Vitamin B6-dependent epilepsy is a rare disease, but it is preventable and treatable, so once detected, it is recommended to seek medical attention as soon as possible, complete relevant tests, and actively cooperate with the doctor’s treatment.