ObjectiveTo investigate the clinical and imaging features of patients with pulmonary lymphangioleiomyomatosis (PLAM),to improve the knowledge of this disease. MethodsEighteen patients with PLAM admitted into Zhongshan Hospital between 2003 and 2013 were retrospectively analyzed. Relevant literatures were reviewed. ResultsAll the 18 cases were female and had dyspnea on exertion. Three of them suffered from chylothorax,6 from pneumothorax and 5 from hemoptysis. The high resolution CT (HRCT) findings in all the 18 cases showed multiple small cysts with thin wall diffusely distributing in the bilateral lung. PLAM was confirmed by biopsy in 14 cases. Pulmonary function tests in 4 cases showed obstructive ventilatory dysfunction. Nine cases (50%) were misdiagnosed as other diseases. All the patients were given symptomatic and supportive treatment. Literatures review showed that as a rare diffuse lung disease,PLAM is more common in women of childbearing age,mainly presenting with dyspnea,hemoptysis,pneumothorax and chylothorax. Diagnosis of the disease relies on medical history,HRCT features and lung tissue biopsy pathology. HRCT examination has diagnostic value for PLAM. Although anti-estrogen treatment has certain effects,rapamycin may be a promising drug for PLAM therapy. ConclusionPatients with PLAM have characteristic clinical and radiographic manifestations. Early diagnosis and effective treatment are keys to the management of patients with PLAM.