Bronchiolitis obliterans organizing pneumonia (BOOP) occurred in a 53-year-old woman with well-documented Sjögren's syndrome (SjS) and polymyositis (PM). BOOP has often been reported as a pulmonary manifestation of collagen vascular diseases, mainly rheumatoid arthritis (RA), but the association of BOOP and PM has rarely been documented. A search of the literature showed only 16 case reports of BOOP associated with polymyositis-dermatomyositis (PM-DM). It is interesting that BOOP occurred prior to PM-DM, while it is commonly believed to occur after RA.