Interstitial lung disease (ILD), a group of diffuse parenchymal lung disorders classified together based on specific clinical, radiologic, and histopathologic features, is often associated with significant morbidity and mortality and is a common manifestation in connective tissue disease (CTD). ILD often arises within the context of a specific exposure or is associated with an underlying CTD. The CTDs are a spectrum of systemic autoimmune disorders with significant clinical heterogeneity characterized by immune-mediated organ dysfunction, and the lung is a frequent target. All CTD patients are at risk of developing ILD, and those with systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM), and rheumatoid arthritis (RA) are at particularly high risk. ILD may develop at any point in the natural history of CTD, is most frequently identified in the setting of an established CTD, and may also be the first clinically apparent manifestation of occult CTD. Determining whether a patient has a diagnosis of CTD-associated ILD is important, as this knowledge may impact treatment decisions, guide surveillance for other concomitant clinical features, and help with assessment of prognosis.