Angioimmunoblastic T-cell lymphoma (AITL) is a distinct subtype of peripheral T cell lymphoma. AITL derived from follicular T helper cells, TET2, IDH2, DNMT3A mutations are frequent in AITL.The typical symptom of AITL is generalized lymphadenopathy, with B symptoms and extranodal invasion, however most patients are at advanced stages when diagnosed. The standard treatment remains to be determined, though the anthracycline-based regimen are most frequently used withunsatisfied long-term outcome. Recently, with the advance on moleculart biology of the pathogenesis of AITL and the booming of immunotherapy and targetted therapy, novelagents and combinedmodalities are constantly emerging. Further clinical investigation of the optimal regimen is needed. This article reviews the current status and research progress on the treatment of AITL.