Lymphomatoid granulomatosis is the only form of pulmonary angiitis histologically characterized by a necrotizing angiocentric and angiodestructive lymphoid infiltrate, with an admixed T-cell reaction. We evaluated three patients with a single lung nodule not diagnosed by routine radiological and endoscopic assays. Our investigations showed a prevalence of T-cells in areas of diffuse infiltration, which were actively replacing reactive follicular areas of B-cells, similarly to T-cell lymphomas. Further pathologic assays suggested the histologic diagnosis of grade I lymphomatoid granulomatosis for all three evaluated specimens. After two years, patients treated with a combination of surgical resection and chemotherapy were disease free, supporting the efficacy of aggressive therapy in the management of this often mistreated group of lymphoid proliferations.