Patients with congenital immunodeficiency (CID) syndromes are susceptible to various microorganisms. However, relatively few CID disorders develop mycobacterial disease. We describe clinical features, laboratory findings and therapeutic outcome of children with CID who had tuberculosis disease. Medical reports of 10 patients were reviewed. Three patients had chronic granulomatous disease, two had common variable immuno deficiency, the others had cyclic neutropenia, combined immunodeficiency, hyperimmunoglobulin E syndrome, selective IgA deficiency and X-linked agammaglobulinemia. Eight patients presented with pulmonary tuberculosis, one had tuberculosis arthritis, one had tuberculosis osteomyelitis. There was acid fast bacilli in sputum of two, bone marrow aspiration in one and postmortem lung biopsy specimen in one patient. Mycobacterium tuberculosis grew in sputum of one and articular fluid aspirate of one patient. One patient was diagnosed with bone biopsy specimens characteristic for tuberculosis. The remaining three patients were diagnosed to have tuberculosis disease as they had positive tuberculin skin test and clinical and radiologic findings unresponsive to nonspecific treatment. All patients were treated with antituberculous drugs. Mycobacterium species may be important pathogenes in children with CID, especially in endemic regions.