Congenital neutropenia is an inherited disorder. The pathogenesis of congenital neutropenia is not clear, but it may be caused by mutations in several genes and is hereditary, with an increased incidence possibly occurring in consanguineous marriages. There are two general types, infantile hereditary agranulocytosis and severe familial neutropenia. Infantile hereditary agranulocytosis is autosomal recessive and severe familial neutropenia is dominant. Congenital neutropenia tends to have a family history, and the presence of a gene for severe congenital neutropenia in the family may lead to an increased chance of developing the disease. It should be treated with radical surgery such as allogeneic hematopoietic stem cell transplantation as soon as possible after detection. If there is a blood count suggestive of neutropenia, prompt medical attention and aggressive treatment are recommended.