We report on a 16 year old girl with a three-year history of systemic lupus erythematosus who developed a case of acute lethal haemorrhagic pancreatitis. She presented with high grade fever, skin rash, malaise, and arthralgias. Laboratory lupus activity parameters were markedly elevated. In the absence of renal, pulmonary, cardiac or cerebral involvement, our patient developed pancreatitis leading to pancreatogenic shock. Until 14 days before the onset of pancreatitis, the patient's medications included prednisolone, azathioprine and methotrexate. At autopsy, no autoimmune vasculitis was found in the affected pancreatic tissue. Therefore, an etiologic role of combination therapy had to be considered. Whereas methotrexate has never been reported to be linked to pancreatitis, a few publications describing prednisolone and azathioprine in connection with pancreatitis do exist. Thus, if pancreatitis is not just termed idiopathic, it must be attributed to a combination regimen of drugs including methotrexate. A review of the literature shows that pancreatitis in SLE is rare and has never been associated with methotrexate therapy before.