© 2021 Wiley Periodicals LLCBackground: Pulmonary involvement which can be infectious or noninfectious is one of the most frequent complications in patients with primary immunodeficiency (PID). Objective: The aim of this study is to assess the pulmonary system of the pediatric patients with PID and report the demographical, clinical, and radiological findings regarding the underlying PID. Methods: The present study included pediatric patients who were receiving immunoglobulin replacement therapy (IGRT) for PID (combined immunodeficiency [CVID] (23), common variable immunodeficiency (15), and agamaglobulinemia ) so far or newly diagnosed and started IGRT at Hacettepe University Immunology Department, between January 2015 and January 2018. Results: A total of 48 patients (60.4% male), aged 9.9 (6.1–14) years were included. Time of delay in the diagnosis of immundeficiency was 2.27 (interquartile range: 1.0–6.75) years. CVID patients exhibited higher respiratory system symptoms, as well as a history of recurrent lung infection and hospitalization. Hilar and mediastinal lymphadenopathies, peribronchial thickening, and bronchiectasis were the most common pulmonary complications and more than three lung lobes were affected in 69%. Among the newly diagnosed patients, bronchiectasis was present in 25% and more than three lobes were affected in 62.5%. Although pulmonary nodules and mediastinal lymphadenopathy were frequently computed tomography findings in our patients, only two patients (4.16%) were diagnosed with interstitial lung disease. Conclusion: Although bronchiectasis is predominantly reported as a long-term complication in adult patients with PID, half of our pediatric patient cohort with PID had bronchiectasis, even the newly diagnosed patients. Long-term follow-up is needed to assess the extent to which these pulmonary complications that develop in the natural course of the disease can be prevented by IGRT.