Immunological disorders predispose to multiple changes in the pulmonary tissue. Frequent complications, such as bronchiectasis, interstitial lung damage, fibrosis, lymphoma are common. This study presents the findings in our 3 patients with severe primary immunodeficiency and lung disease. The first patient with both CVID and benign lymfoproliferative disorder, the second with both CVID and extrinsic allergic alveolitis recognised, the third – with udetectable IgA, low subclasses IgG1 i IgG3 had reticular pattern in chest HRCT scans. Prognosis is uncertain in both primary immunological disorder, as well as interstitial lung diseases. Early recognize of the dual presentation of primary immunological disorder and lung diseases and adequate treatment, will prevent irreversible structural lung damage.