Acute pancreatitis is an uncommon manifestation of systemic lupus erythematosus (SLE). In most reported cases, it occurs in the setting of SLE with multi-organ involvement. We report an unusual case of SLE in a Sri Lankan woman presenting with acute pancreatitis with the pancreas as the sole major organ affected. The diagnosis of acute pancreatitis was based on clinical features, serum and urinary amylase levels and computerised tomography. She responded well to high-dose corticosteroid. We also review the literature and discuss the prevalence, theories of pathogenesis and treatment of this condition.