Abstract: Connective tissue disease (CTD) is a group of systemic disorder characterized by autoimmunity and autoimmune-mediated organ and system damage. The pulmonary complication is an important extra-articular feature of CTD and a major cause of mortality. The key point in the management of CTD-associated interstitial lung disease (CTD-ILD) should focus on the type of ILD, the stage of the disease, and what proper measures should be taken. CTD-ILD is still using the classification of idiopathic interstitial pneumonia. However, there is no authoritative clinical guidance for the diagnosis and treatment of CTD-ILD. More and more studies show that some auto-antibodies are strongly associated with CTD-ILD, which may guide clinical practice. In clinical practice, physicians should particularly pay more attention to CTD patients with insignificant pulmonary manifestations, so as to identify CTD-ILD in the early stage and intervene at the right time to delay the progression.