As the use of high-dose intravenous gamma globulin and available antimicrobials expands, the incidence of noninfectious common variable immunodeficiency–related disorders, such as granulomatous-lymphocytic interstitial lung disease (GLILD), is likely to increase. A thorough clinical evaluation using a multidisciplinary care model is recommended to achieve accurate diagnosis of GLILD. Enough progress has been made in the understanding of GLILD over the last decade that clinicians are now poised to conduct therapeutic trials. Data from large registries and population-based studies of GLILD are needed to better understand the disease trends and burden. This article focuses on the diagnosis and management of GLILD.