We report a case of a 64-year-old man with chronic diarrhea who was diagnosed of a systemic reactive (AA) amyloidosis associated with a jugular paraganglioma. This neoplasm was diagnosed 30 years previously and it was not removed after extensive evaluation by a multidisciplinary team. Chronic inflammatory diseases are the major cause of AA amyloidosis. However, benign tumors, such as jugular paragangliomas, are considered to be one of the tumors that can result in AA amyloidosis.