The objective of this study was to describe the pulmonary abnormalities on high-resolution computed tomography (CT) in patients with mixed connective tissue disease (MCTD). The study included 41 patients who met the diagnostic criteria for MCTD and showed abnormal findings on high-resolution CT. The presence, extent, and distribution of various high-resolution CT findings were evaluated. The predominant abnormalities included areas of ground-glass attenuation (n = 41), subpleural micronodules (n = 40), and nonseptal linear opacities (n = 32). Other common findings included peripheral predominance (n = 40), lower lobe predominance (n = 39), intralobular reticular opacities (n = 25), architectural distortion (n = 20), and traction bronchiectasis (n = 18). Less common findings included honeycombing, ill-defined centrilobular nodules, airspace consolidation, interlobular septal thickening, thickening of bronchovascular bundles, bronchial wall thickening, bronchiectasis, and emphysema. Pulmonary involvement of MCTD is characterized by the presence of ground-glass attenuation, nonseptal linear opacities, and peripheral and lower lobe predominance. Ill-defined centrilobular opacities were uncommonly seen.