Abstract:Bacillus-Calmette Guérin (BCG) vaccine is a live attenuated strain of Mycobacterium bovis (M.bovis) subsp. Intradermal BCG vaccination can prevent severe types of tuberculosis in children such as milliary tuberculosis of lung or tuberculous meningitis. Children with several primary immunodeficiencies are susceptible to BCG vaccines. The prevalence, pathogenesis, clinical manifestations for severe combined immunodeficiency, chronic granulomatous disease, and Mendelian susceptibility to mycobacterial disease will be reviewed. More attention will be paid on features, treatment, and prognosis of BCG diseases in children with such primary immunodeficiencies mentioned above.