Abstract: Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor of uncertain histogenesis with a nonrecipro-cal translocation der (17) t (X;17) (p11.2; q25) leading to the fusion of the ASPL gene to the gene encoding transcriptionfactor TEF3. ASPS is mainly found in the extremities, especially the deep soft parts of lower limbs. Histologically, the tu-mor shows typical alveolar and nest arrangement with eosinophilic granules or crystals in the cytoplasm of tumor cells.ASPS often occurs in young women. Although it displays a slow clinical course, it often metastasizes to the lung, bone,liver and brain, worsening the prognosis. Complete resection is critical for treatment of localized alveolar soft part sarcoma.Adjuvant chemotherapy and radiotherapy have demonstrated few benefits. Here, we present a case of primary ASPS of theleft leg with lung and brain metastases in a 37yearold woman. She underwent surgery, radiotherapy and interventionaltherapy. Because of the progression of metastases in her lungs she was also given immunotherapy, molecular target therapyand traditional Chinese medicine. We investigate whether the modern treatment can prolong the survival of ASPS patients.