Sjögren's disease (SjD) is characterized by immune-mediated glandular dysfunction and can occur in isolation or accompany other autoimmune rheumatic disorders. Although sicca symptoms are the principal manifestation of SjD, it is the extraglandular manifestations, particularly in the respiratory system, that may be the more significant contributor to morbidity and mortality. Almost all compartments of the respiratory system have been described in association with SjD, including (1) the airways, with xerotrachea that may lead to chronic cough, bronchiolitis, and bronchiectasis; (2) the lung parenchyma, with both fibrotic and cystic lung diseases; (3) the pulmonary vasculature, with pulmonary hypertension; and (4) lymphoproliferative complications such as amyloidosis, light-chain deposition disease, and mucosa-associated lymphoid tissue (MALT) lymphomas. Depending upon the modalities used for screening, these abnormalities can be present in the majority of patients with SjD, some of whom are asymptomatic. Thus, awareness from both rheumatology and pulmonary specialists is essential to facilitate early detection, using available consensus guidelines to direct appropriate screening and monitoring strategies. Although specific clinical trials for patients with pulmonary manifestations of SjD are lacking, the extrapolation of the literature from similar pulmonary complications of other rheumatic diseases is available to guide the clinician when intervention is needed.