Pulmonary manifestations are common in connective tissue diseases, and are associated with significant morbidity and mortality in this patient population. Systemic sclerosis (SSc) and mixed connective tissue disease (MCTD) are clinical entities for which the detection of lung involvement is essential to improve patient care and outcomes. This article discusses the pathogenesis, clinical presentation, and evaluation of the patient with pulmonary disease related to SSc and MCTD, with an emphasis on interstitial lung disease and pulmonary hypertension.