Acute pancreatitis is a rare, but fatal, manifestation of systemic lupus erythematosus. Only 10 systemic lupus erythematosus-associated pancreatitis cases were found in a search of published articles. We report a 24-year-old woman without significant medical history, who was admitted with abdominal pain, nausea and vomiting, which was diagnosed as pancreatitis. It was discovered to be the initial presentation of systemic lupus erythematosus. The first time she was admitted, she recovered with conservative management and steroid therapy. Two months later, she was readmitted to our hospital with symptoms and signs of acute abdomen, which was attributed to her discontinuation of the therapeutic regimen with corticosteroids just after her previous discharge. She underwent laparotomy twice for signs of peritonitis. Despite administration of a monoclonal antibody, rituximab, she died due to the progression of systemic lupus erythematosus activity.