Macroglobulinemia has been reported in association with malignant lymphoma and chronic lymphatic leukemia on many occasions, and pulmonary lesions have been described in Waldenstrom's macroglobulinemia. We record here the first case of macroglobulinemia associated with a primary pulmonary lymphoma. Biopsy material from the lung was examined by light microscopy, immunofluo‐rescence, and electron microscopy. This correlated study showed the tumor to present a moderately pleomorphic pattern with lymphocytoid and plasmacytoid cells interspersed with reticulum cells, mast cells, and immature blast cells. Many of the tumor cells were actively engaged in protein production and had a well‐developed endoplasmic reticulum. Macrophages with complex lysosomal bodies were identified, and these could be correlated with globular bodies seen on light microscopy, and with cytoplasmic inclusions seen by im‐munofluorescence. Immunoelectrophoresis of the serum identified an I(MK monoclonal component. Clinical remission and regression of the lung mass has been obtained with continuous chlorambucil therapy.