Primary antibody deficiency diseases result from a genetic defect that causes misfunction of 1 or more of the immune system elements. Due to the increased awareness among physicians and the success of new treatment modalities, the number of pediatric patients reaching adult age and the number of patients diagnosed in adult age is increasing. Adult patients comprise more than half of the total cases. Primary antibody deficiencies are the most common immunodeficiency type in adults, and these may cause recurrent upper and lower respiratory tract infections and result in the development of bronchiectasis. Among non-infectious pulmonary complications, any type of interstitial lung disease may be seen; however, a special type seen in patients with common variable immunodeficiency, namely granulomatous lymphocytic interstitial lung disease, is the one most commonly investigated. Underlying or accompanying immunodeficiency may be present in patients with asthma and chronic obstructive pulmonary disease, especially if the disease requires frequent hospitalizations and/or is severe. Early diagnosis and appropriate management of primary antibody deficiency diseases in patients with respiratory symptoms are crucial to decrease complications and increase survival.