First reported by Swedish pediatrician Kostmann in 1956, it is also known as Kostmann syndrome.[1] At present, the disease has been confirmed to be associated with a variety of gene mutations, mainly occurring in genes such as ELANE, HAX1, GFI-1, WASP, G6PC3, CSF3R, and JAGN1, of which 50% to 60% of cases are caused by ELANE gene mutations.[2,3] Clinically, the incidence of SCN is extremely low, and children often have repeated serious bacterial infections soon after birth, which will pose a threat to their life and health without proper treatment. The gene discussed is ELANE; the disease is severe congenital neutropenia.