Bronchiolitis obliterans organizing pneumonia (BOOP) is characterized histologically by formation of granulation-tissue plugs within the lumens of small airways. It was reported in associations with various disorders including infection, drug reactions, and collagen vascular diseases. However, there are only rare reports of BOOP accompanied with systemic lupus erythematosus (SLE), especially in pediatric population. Herein, authors report a case of an adolescent with SLE who initially manifested respiratory illnesses due to BOOP. A 14-year-old girl referred to our clinic for protracted cough with fever, dyspnea, and hemoptysis. Her chest X-ray revealed predominant multifocal consolidations in bilateral lung fields with pleural effusion. Computed tomography (CT) scan showed surrounding ground glass opacities with crazy paving appearance with multiple centrilobular nodules suggesting diffuse alveolar hemorrhage. Laboratory tests revealed pancytopenia, elevated blood urea nitrogen and creatinine, low serum levels of complements and positivity for antinuclear antibody, p-anti-neutrophil cytoplasmic antibody, and anti-double stranded DNA antibody. To exclude the possibilities of vasculitis and other mixed connective tissue diseases, video assisted thoracic surgery biopsy was performed. The pathology confirmed BOOP and systemic steroid therapy including high dose methylprednisolone commenced. Her respiratory symptoms, pulmonary function tests, and radiologic findings showed significant improvement following treatment, and the patient is now doing well without a relapse. This pediatric case of BOOP as the initial presentation of SLE brings the need of more attention to the pulmonary manifestations of SLE in children.