This article provides a broad overview of the complex intersection between the connective tissue diseases (CTDs) and their pulmonary manifestations. Indirect pulmonary complications – such as respiratory infection and medication-induced lung toxicity – are briefly discussed, and the importance of a comprehensive assessment of the patient with CTD with respiratory symptoms is emphasized. A concise review of the many pulmonary manifestations of each specific CTD is provided, and particular emphasis is placed on CTD-associated interstitial lung disease.