Intravenous immunoglobulins have been widely used for the treatment of primary immunodeficiency diseases for more than 20 years, but they are expensive and not curative. In contrast, HLA-identical HSCT and gene therapy are the only effective ways to cure most primary immunodeficiencies. Hematopoietic stem cell transplantation for primary immunodeficiency diseases is now widely practiced in developed countries. Hematopoietic stem cell transplantation is mainly applied in two areas: immunodeficiencies of the lymphatic lineage, such as hyper IgMemia, severe combined immunodeficiency and eczematous thrombocytopenia with immunodeficiency syndrome; and immunodeficiencies of the myeloid lineage, such as chronic sarcoidosis and leukocyte adhesion defects. In the case of our country, there are still numerous problems for primary immunodeficiency diseases. For example, screening and registration are not yet sound nationwide or locally; molecular biology diagnosis is significantly lagging behind; some children cannot adhere to standardized treatment because of the high cost of treatment; and most children with primary immunodeficiency diseases die prematurely without bone marrow transplantation due to failure to identify cases in a timely manner, lack of adequate donor sources and insufficient attention from society. With the significant increase of cases of primary immunodeficiency diseases found in China, it is important to draw the attention of society and the medical community. Currently, developed countries abroad have established corresponding associations or organizations for primary immunodeficiency diseases, calling on the whole society to care for these patients.