Vitamin B6-dependent epilepsy is an autosomal recessive disorder that has been recognized in humans for more than 40 years. The disease occurs in infants and young children and has a variety of clinical manifestations with the following characteristics: 1. The seizures are initially effective on anticonvulsant drugs but eventually develop into refractory epilepsy; 2. Vitamin B6-dependent epilepsy reoccurs 6 weeks after successful discontinuation of phenobarbital for neonatal convulsion control; 3. The interictal period is extended to 5.5 months and seizures reoccur after discontinuation of vitamin B6; 4. seizures cannot be controlled by vitamin B6 supplementation for 8 months, but later seizures can be successfully controlled. With the increasing understanding of the disease and the increasing number of neurodiagnostic methods, the detection rate of the disease will increase compared to the previous ones, so it is proposed that the diagnosis of the disease should be considered in children up to 18 months of age with seizures and one or more of the following conditions: 1. past history, pregnancy history and perinatal normal infants with cryptogenic epilepsy; 2. siblings with severe convulsive disorders and often died due to persistent epilepsy 3. those who present with persistent focal seizures or unilateral seizures with incomplete impairment of consciousness; 4. those with aura symptoms such as irritability, restlessness, crying or vomiting before onset. The neurochemical basis of the disease is not well understood. IQ assessment, neurobiochemical evaluation, and functional brain imaging findings may provide guidance for longitudinal assessment and management of the disease.