LAM has the following clinical features: 1, most cases are women of childbearing age; 2, spontaneous pneumothorax; 3, celiac disease (pneumothorax and celiac disease can recur); 4, some patients have a small amount of hemoptysis; 5, dyspnea; 6, hypoxemia, obstructive or mixed ventilation dysfunction; 7, HRCT shows diffuse cystic changes in both lungs; 8, extra-pulmonary manifestations (renal and retroperitoneal changes) may be present; 9. Surgical lung biopsy can confirm the diagnosis, and a positive HMB45 antibody associated with melanoma is a diagnostic marker for LAM. The main manifestations of this disease are pneumothorax, celiac disease and diffuse cystic changes in both lungs. The differential diagnosis needs to be considered in the light of these aspects. However, when pneumothorax or celiac disease is present in conjunction with diffuse cystic type lesions in both lungs, the possibility of LAM needs to be highly suspected. Diffuse lesions in both lungs need to be differentiated from a number of diseases such as: emphysema, idiopathic interstitial pulmonary fibrosis (honeycomb lung), cystic bronchiectasis, pulmonary Langerhans’ granuloma, and pulmonary cystic fibrosis.