Objective To discuss the clinical features of Lymphangioleiomyomatosis. Methods One case of a pulmonary associated with retroperitoneal lymphangioleiomyomatosis was retrospectively analyzed. Analysis of clinical characteristic and the present situation of diagnosis and treatment of the disease was made combined with the literature. Results The disease mainly occurs in child-bearing women. The main clinical manifestations are pant, chylothorax, pneumothorax, and haemoptysis,etc. Lung function was impaired, retroperitoneal lesions were common extrapulmonary . CT showed thin-walled capsule sample change with same size and uniform distribution, liquid pneumothorax and grinding glass sample change can be associated. Abdominal CT demonstrated retroperitoneal cystic-solid tumors with lymph node enlargement. Conclusion LAM should be considered when patients have corresponding respiratory symptoms, accompanied with typical lung and abdominal CT performance. pathologic examination has specificity.