Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening dermatological conditions that are characterized by mucosal erosions, epidermal detachments and erosions. The most common causes of SJS and TEN are drugs; other causes such as systemic lupus erythematosus (SLE), vaccinations and infections have been rarely implicated. We present the case of a 14-year-old female patient with acute pancreatitis as an initial manifestation of systemic lupus erythematosus, complicated by the toxic epidermal necrolysis with a fatal outcome. She initially presented with abdominal pain, fever, vomiting, and intolerance to oral intake and elevated pancreatic enzyme levels. Systemic lupus erythematosus was diagnosed secondary when her condition has been already complicated by the toxic epidermal necrolysis. The administration of corticosteroids and high doses of intravenous immunoglobulin did not lead to positive effects in the treatment of our patient.