Objective To investigate the clinical features, imaging manifestations, treatment response and prognosis of children with systemic juvenile idiopathic arthritis associated lung disease (sJIA-LD). Methods The clinical data of 15 children with sJIA-LD were retrospectively analyzed, and the clinical characteristics, laboratory parameters, imaging changes and treatment prognosis were summarized. Results There were 7 boys and 8 girls in current study and the average age was 7 years. The common clinical symptoms were fever (15 cases), joint swelling and pain (12 cases), cough (12 cases), rash (11 cases), lymph node enlargement (9 cases), serositis (8 cases), hepatosplenomegaly (8 cases), shortness of breath (7 cases), chest pain (2 cases) and abdominal pain (1 case). Seven patients were complicated with macrophage activation syndrome (MAS). All the 15 children underwent chest high-resolution CT examination, and the results showed strip or patchy shadow in 15 children, consolidation shadow in 3 children, mesh shadow in 2 children, nodular shadow, calcification shadow and atelectasis in 1 child each. The commonest lesion site was the left lower lobe of the lung (13/15). The imaging findings revealed 9 cases of organizing pneumonia, 5 cases of non-specific interstitial pneumonia, and 1 case of usual interstitial pneumonia. All the 13 patients were treated with non-steroid anti-inflammatory drug and glucocorticoids. In the diagnosis of sJIA-LD, 8 cases were treated with slow-acting antirheumatic drugs, including 3 cases of cyclosporine, 4 cases of methotrexate and 1 case of cyclophosphamide. Eight children were treated with tocilizumab. The condition of 12 children improved, and chest high-resolution CT showed improvement. One patient had lung disease progression and eventually died. Conclusion The early clinical symptoms of sJIA-LD are mild but the mortality is high. The high-resolution CT is helpful for early diagnosis.