To explain these issues, we conducted a systematical review and a meta-analysis from all eligible studies, in order to provide more exact estimate of the association among MTR c.2756A>G (rs1805087), MTRR c.66A>G (rs1801394), TC2 c.776C>G (rs1801198), CBS c.844ins68, and MTHFD1 c.1958G>A (rs2236225) polymorphisms and the maternal risk for DS. The gene discussed is CBS; the disease is Dravet syndrome.