In this study, we report three cases diagnosed with human T-cell lymphotropic virus type 1 (HTLV-1) and Sjögren’s syndrome (SS) who developed pulmonary complications. Radiologic and pathologic findings were evaluated. Although the histologic diagnosis was considered to be hypersensitivity pneumonitis in case 1, and discordant usual interstitial pneumonia (UIP) in cases 2 and 3, lymphocytic alveolitis was observed in all cases. We also did a literature review and concluded that, although the pathologic diagnosis of pulmonary complications in HTLV-1 carriers with SS may vary, lymphocytic infiltrations are commonly observed.