Objective To investigate the clinical characteristics of spontaneous pneumothorax secondary to systemic lupus erythematosus (SLE). Methods A case of spontaneous pneumothorax secondary to SLE was reported and related literatures were reviewed. Results A female patient, 26 years old, complaining of repeated fever for 3 months, chest tightness and shortness of breath more than 10 days, was hospitalized in December 2016. After admission, the patient was diagnosed as acute lupus pneumonia and received glucocorticoid, cyclophosphamide and plasma exchange therapy. Sudden right chest pain happened during hospitalization. Bedside chest CT showed right pneumothorax. After closed thoracic drainage, the syndrome of the patient was improved. A total of 14 patients were reviewd but 2 patients were excluded because of incomplete data. The left patients included 9 females and 3 males. The mean age was (28.0±11.9) years. Clinical manifestations of spontaneous pneumothorax secondary to SLE were sudden chest tightness and shortness of breath accompanied by subpleural lung cavity and vesicular changes. The majority of patients could be cured with drainage. Conclusions Pneumothorax secondary to SLE is rare which is associated with the rupture of cavity and cyst in pulmonary secondary to SLE. Majority of patients with pneumothorax could be cured after closed thoracic drainage.