Angioimmunoblastic lymphadenopathy with disproteinemia (AILD) is a rare lymphoproliferative disorder. The clinical course varies and about 15–20% of the patients develop a malignant lymphoma. The association of AILD and carcinoma is very rare: only 4 cases have been reported in the literature. They are reviewed here and 1 case is described. This case is of special interest for the following reasons: (1) the exceptional length of time which elapsed between the diagnosis of AILD and the onset of an adenocarcinoma of the colon (118 months); (2) the long survival (the patient is still alive and well 140 months after the diagnosis of AILD); (3) unlike the cases reviewed, in our patient the carcinoma was diagnosed when AILD was in clinical remission, so a radical treatment was possible. In the cases previously reported, carcinomas arose in the lung (2 patients), pancreas (1 case), and stomach (1 case). The rare association of AILD and carcinoma is probably coincidental; however, the growth of solid tumors of nonlymphoid nature may be related to the impaired T cell function, and should be kept in mind in the management of patients with AILD.