Abstract: Alveolar soft part sarcoma is a rare malignant tumor. Although there are more than 60 years of experience in clinical treatment, the tumor’s histological origin, risk factors, clinical progression and the best treatment scheme are not very clear. In the past 10 years, many scholars have carried out a lot of research on this tumor. In this review, we discussed recent publications related to alveolar soft part sarcoma in order to better understand and search for new ways to treat this disease with targeted therapy and immune checkpoint inhibitor therapy.