Systemic amyloidosis is a disorder characterized by extracellular deposition of amyloid in various organs and tissues including the kidney, heart, and liver. However, pancreatic involvement is rare, and has not been reported in Korea. Systemic amyloisosis involving pancreas needs to be differentiated from several pancreatic diseases because of diffuse pancreatic enlargement and partial stricture or obstruction of main pancreatic duct. Recently, we experienced a 60-year old man who was suspected as autoimmune pancreatitis or infiltrative disorders on imaging studies, and finally diagnosed as systemic amyloidosis involving pancreas and liver on biopsy examination. We report the case with review of the relevant literatures.