Systemic lupus erythematosus (SLE) is an autoimmune disease characterised by the presence of several autoantibodies, immune complex formation and multiple organ system involvement. SLE has a wide range of manifestations involving nearly all organ systems. Hypertriglyceridemia (HTG) in SLE is a well-established and a common abnormality, which is generally mild and not included in the diagnostic criteria of SLE. HTG as the initial manifestation of SLE in adult patients in association with acute pancreatitis at levels below 1000 mg/dL has not been previously reported. Here, we report a case of rare presentation of moderate HTG (TG-869 mg/dL) with pancreatitis at disease onset in an adult women which later proved to be due to SLE and progressed to levels of severe HTG during the course of illness. The patient was successfully treated with plasma exchanges and cyclophosphamide.