Considering the functional effects of the 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, it is expected that these polymorphisms may be associated with the maternal DS risk and several studies have been carried out to determine this association. This evidence concerns the gene MTHFD1 and Dravet syndrome.