An autopsy case with pulmonary lesions analogous to Liebow's lymphomatoid granulomatosis (LYG) and some other unusual pictures were reported. A 29-year-old male who had been diagnosed and treated as Sjögren's syndrome for eleven years because of intermittent swelling of the parotids with diffuse lymphoid cell infiltration and positive serum RA-test, showed several circumscribed densities on the chest X-ray examination which were deemed to be of interlobar pleurisy. On autopsy, the nodular lesions in the lung were composed of angiocentric-angiodestructive lymphoid cell proliferation analogous to LYG. In addition, the present case was characterized by focal glomerulonephritis and splenic trabeculitis which are quite unusual associations in LYG. The relationships of the present case to other similar diseases such as Wegener's granulomatosis, Sjögren's syndrome, primary lymphoma of the lung, etc. were discussed.