A new clinico-pathologic disease of Iymphoid system has been reported as immunoblastic Iymphadenopathy by Lukes and Tindle and as angioimmunoblastic Iymphadenopathy with dysproteinemia (AILD) by Frizzera et. at. Clinically it occurs most commonly in elder person is characterized by acute onset of constitutional symptoms, generalized Iymphadenopathy, hepatosplenomegaly and immunologic abnormalitis such as consistent polyclonal gammopathy and often hemolytic anemia. Histologically it has a diagnostic triad ; 1) loss of nodal architecture and pleomorphic infiltration of immunoblasts, plasmacytoid immunoblasts, plasma cells and Iymphocytes, 2) prominent proliferation of arborizing small vessels and 3) deposit of PAS-positive amorphous material in the interstitium. We report here a case of angioimmunoblastic Iymphadenopathy with dysproteinemia of a 64 year old male patient and review the literature.