Pulmonary hemorrhage (PH) is a rare but potentially life-threatening manifestation of systemic lupus erythematosus (SLE). In this report we describe a 13 year old girl with PH as the sole presenting clinical manifestation of her SLE. Her serology was diagnostic of SLE and one year after presentation she developed arthritis. She had a rapid serologic but delayed clinical response to combination therapy of intravenous pulse methylprednisolone, pulse cyclophosphamide and daily prednisone. Awareness of the possibility of pulmonary hemorrhage as a presentation of SLE may aid in the diagnosis and early, aggressive management of this condition.