Abstract: Objective　To explore the diagnosis and treatment of neonatal convulsion. Method　The clinical data of neonatal convulsion in two cases were retrospectively analyzed. Results　Two children were both males, and one was premature. Convulsions occurred on the second day after birth. The gene detection was performed after the common causes of convulsions were excluded after admission. The SCN2A gene mutation (c.5558A>G, p.H1853R) was found in case one and the treatment with luminal and topiramate had no obvious effect. The KCNQ2 gene mutation (c.836G>A, p.G279D), a novel mutation, was found in case two. Initially luminal was orally administered. Keppra and topiramate were added on 21st and 36th day respectively after birth and their dosages were increased gradually. The muscle tension of the lower extremities is still slightly higher. Conclusion　The etiology of neonatal convulsion is complex. Gene detection can identify the cause and provide a reference for the treatment and prognosis evaluation.