Pulmonary symptoms as the initial or primary manifestation of SLE are rare. When pulmonary symptoms are present, they occur most commonly when other organ systems are involved. The absence of skin and renal involvement, the presence of normal serum complement, and the poor response to corticosteroids in this patient are of interest. Pulmonary disease produced by childhood SLE may represent, as in adults, a subgroup of SLE disease. Our report emphasizes the importance of recognizing diffuse interstitial infiltrates as an initial symptom of childhood SLE even in the absence of more obvious signs.