defects of neutrophil function and/or differentiation, defects of motility, defects of respiratory burst, and Mendelian susceptibility to mycobacterial diseases could be classified as main diseases in the category of phagocytes defects. Severe congenital neutropenia, cyclic neutropenia, glycogen storage disease type 1b, p14 deficiency, Barth syndrome, Cohen syndrome, and poikiloderma with neutropenia are primary immunodeficiency diseases with neutrophil function/differentiation defects. Leukocyte adhesion deficiency (LAD types I-III), Rac2 deficiency, b-actin deficiency, localized juvenile periodontitis, Papillon–Lefevre syndrome, specific granule deficiency, and Shwachman–Diamond syndrome are classified in group of motility defects. Chronic granulomatous disease (CYBB, CYBA, NCF1, NCF2, NCF4) is the prototype of defects of respiratory burst. Mendelian susceptibility to mycobacterial diseases predispose individuals to mycobacterium. Mutations in several gene loci have been detected for MSMD, including IL-12RB1, IFNGR1, IFNGR2, IL12B, STAT1, CYBB, IRF8, and ISG15. GATA2 deficiency, pulmonary alveolar proteinosis along with autosomal recessive form of IRF8 deficiency are other diseases that have been classified as phagocytes defects.