Primary immunodeficiency diseases (PIDs) are inherited defects of the innate or adaptive arms of the immune system that differs from secondary immunodeficiencies. Respiratory disorders are significant causes of morbidity and the leading causes of death (30% -65%) in both children and adults with PIDs. The spectrum of respiratory manifestations is extremely wide due to PIDs. PIDs are broadly classified according to the components of the immune system that is primarily disrupted. Predominant antibody deficiency disorders are the most frequent and comprise approximately 70-75% of all PIDs.