Primary immunodeficiencies (PID) are a diverse group of hereditary diseases leading to the impaired immune response that creates high susceptibility to mycobacterium infection. High susceptibility to mycobacterial infections of patients suffering from defects of phagocytosis and combined immunodeficiencies can be explained by predominant participation of macrophages and T lymphocytes in the specific immune response. Mycobacterium tuberculosis, Bacille Calmette-Guerin, and non-tuberculosis mycobacterium (NTM) may cause a severe disease in patients with PIDs. We report here our results of the clinical features of mycobacterium infection presentations in 36 patients with various PIDs.