A case of immunoblastic lymphadenopathy and systemic amyloidosis in a 21-year-old man is presented. The basic defect in immunoblastic lymphadenopathy is believed to be an abnormal immune reaction, most probably of the B cell system. Although systemic amyloidosis is common in some disorders of the B cell system, no cases of systemic amyloidosis complicating immunoblastic lymphadenopathy have been previously reported. Immunoblastic lymphadenopathy includes elements known to be associated with amyloidosis and we believe that this association is not merely coincidental.