Here, we report two pathogenic mutations in <i>ELANE</i>, namely, c.607G>C (p.Gly203Arg) and a novel variant c.416C>G (p.Pro139Arg), found in two Mexican families ascertained via patients with congenital neutropenia who responded positively to the granulocyte colony-stimulating factor (G-CSF) treatment. Here, CSF3 is linked to severe congenital neutropenia.