Pulmonary sarcomatoid carcinoma (PSC) is a pathological subtype of non-small cell lung cancer. Although the incidence of PSC in lung cancer is very low, it is an aggressive cancer, leading to a poor prognosis. Currently, there is no standard treatment for advanced PSC. Targeted therapy can be used for patients with MET exon 14 mutations and patients with other driver gene mutations may also benefit from treatment. The emergence of immune checkpoint inhibitors also provides potential options for advanced PSC treatment, but more clinical data is needed. Additionally, more research may be warranted to explore the effects of chemotherapy, radiotherapy and antiangiogenic therapy. In this review, the authors summarize the research regarding the treatment of advanced PSC.