Purpose of Review <p>Systemic therapy is particularly important for patients with recurrent or incompletely gross-total resected ependymoma. This study focuses on summarizing the systemic therapeutic strategies including molecular targeted therapy, immunotherapy and conventional chemotherapy in recent years, with a view to bringing more advanced treatment options to patients with ependymoma.</p> Recent Findings <p>In studies of systemic therapy for ependymoma, chemotherapy, although controversial, remains effective in patients with recurrent ependymoma. In targeted therapy, tyrosine kinase inhibitors, epigenetic agents and PARP inhibitors have shown promising antitumor activity in preclinical studies and some clinical trials. In immunotherapy, strategies such as immunological checkpoint inhibitors and CAR T cells therapy have shown potential therapeutic value in specific patient populations.</p> Summary <p>Chemotherapy remains an important option for patients with recurrent ependymoma, and recent studies have revealed the great potential of targeted therapy and immunotherapy, suggesting that further in-depth studies on the optimization and combined application of targeted drugs and immunotherapy strategies, etc., can be conducted in the future.</p>