STXBP1-encephalopathy is a severe neurodevelopmental disorder caused by heterozygous pathogenic variants in the STXBP1 gene, characterized by early-onset refractory epilepsy and severe developmental delay.[10] This study systematically analyzed the clinical and genetic characteristics of 19 Chinese children with STXBP1-encephalopathy, identified 7 novel pathogenic variants, and confirmed the good efficacy of LEV, which provides important clinical and genetic data for the diagnosis and treatment of this disease in Chinese populations. Here, STXBP1 is linked to Encephalopathy.