Abstract： Objective To analyze the pathogenesis,clinical features,diagnosis and differential diagnosis of primary perivascular epithelioid cell tumor(PEComa).Methods The clinical features,auxiliary examinations and diagnosis of a case with rapidly progressive pulmonary malignant PEComa were reported and the related literatures were reviewed.The literature review was carried out respectively in Wanfang Data,CNKI and PubMed from Jan.1975 to Jul.2015 with" pulmonary malignant perivascular epithelioid cell tumor" and" PEComa" being the search terms.Results A 50 year-old female patient was admitted to the hospital on September 4,2014 because of cough and dyspnea for 60 days,hemoptysis for 40 days and fever for 7 days.Chest CT scan showed diffuse small nodules with infiltrative border and multiple pure and mixed ground-glass opacity.Transbronchial lung biopsy (TBLB) was performed and the pathological study confirmed the diagnosis of primary pulmonary malignant PEComa.The patient declined further specific therapy,but followed by rapidly progressive respiratory failure,and died two weeks after the diagnosis.A total of 8 literatures were retrieved from Wanfang Data,CNKI and PubMed and all of them were case reports.There were 3 male and 5 female patients,aging from 50 to 79 years.Radiographically,the previously reported cases presented as round and well-circumscribed masses with or without multiple nodules in both lungs.The symptoms had no specificity.Conclusions Pulmonary malignant PEComa is a rare disease.It is easily misdiagnosed because of non-specific clinical and imaging manifestations.The final diagnosis depends on pathological biopsy.TBLB is an effective diagnostic method.