This article describes two cases of malignant lymphoma in which the finding of a striking epithelioid granulomatosis was the main clinical manifestation at the outset of the patients' history. Despite some suggestive clinical and laboratory data, the final diagnosis of lymphoproliferative disorder was made only after repeated histological studies in both patients. Although the association between a severe granulomatous reaction and a B-cell neoplasm is rare, the latter should be taken into account as a possible primary disease. The pathogenetic mechanisms postulated by the literature as the cause of granulomatous reactions in lymphoproliferative disorders are reported and discussed in relation to the two cases.