We report a case of systemic lupus erythematosus that concomitantly occurred with autoimmune pancreatitis. The clinical manifestations of pancreatitis improved in response to steroid therapy. Although the pathogenesis of autoimmune pancreatitis is still controversial, as is that of systemic lupus erythematosus, the observations in the present case suggest the presence of an autoimmune mechanism underlying autoimmune pancreatitis. © Japan College of Rheumatology and Springer-Verlag Tokyo 2004.