Systemic lupus erythematosus presenting as acute pancreatitis is rare. We report a case of a 12-year-old girl with a 1-year history of systemic lupus erythematosus who developed active pancreatitis. The pancreatitis was first manifested by nausea, vomiting, fever, and abdominal pain. Elevated serum amylase (578 U/L) and lipase levels (5588 U/L), and pancreatic enlargement on ultrasound and computerized tomography confirmed the diagnosis. She responded well to high-dose corticosteroid. The high titer of antinuclear antibodies (1:1280) and low level of complement components (C3, 42.9 mg/dL; C4, 2.3 mg/dL) during the pancreatitis attack suggested that the pancreatitis may have been due to systemic lupus erythematosus exacerbation and not related to drug therapy.