Summary: Primary immunodeficiencies (PID) are rare, congenital disorders of the immune system. Among them, primary antibody deficiencies (PADs), with CVID, are most often recognized. CVID symptoms are non-specific, encompassing recurrent infections, autoimmune disorders, lymphoproliferation and non-infectious complications such as interstitial lung disease (ILD). The most common form of PID-related ILD is granulomatous-lymphocytic ILD (GLILD), associated with lympho-cytic infiltration and/or granuloma in the lung. Chest computed tomography (CT), lung function tests, bronchoscopy and lung biopsy should be performed among suspected patients. The paper describes the diagnostic criteria, differential diagnosis and treatment based on the data available in the literature. Moreover, the authors emphasize the multi-system nature of GLILD as well as its impact on mortality in the group of patients with immunodeficiency complicated by this diagnosis.