A case of angioimmunoblastic T-cell lymphoma(AITL) which was misdiagnosed as adult Still’s disease was presented.The clinical and laboratory characteristics of this case and related literatures were analyzed and reviewed. The patient was finally diagnosed as AITL(Ann Arbor classification:StageⅢB) secondary to cranial astrocytoma (WHO classification:StageⅢ),complicated with severe pulmonary infection because of long time treatment of corticosteroid and misdiagnosis(about one and a half year).It is concluded that AITL is a rare disease which was easily misdiagnosed.The diagnosis of AITL should combine the clinical manifestation with pathological biopsy as well as corresponding immunohistochemical tests. A case of angioimmunoblastic T-cell lymphoma (AITL) which was misdiagnosed as adult Still’s disease was presented.The clinical and laboratory characteristics of this case and related literatures were analyzed and reviewed. The patient was finally diagnosed as AITL (Ann Arbor classification: Stage IIIB ) secondary to cranial astrocytoma (WHO classification: Stage III), complicated with severe pulmonary infection because of long time treatment of corticosteroid and misdiagnosis (about one and a half year). It is that that AITL is a rare disease which was easily misdiagnosed.The diagnosis of AITL should combine the clinical manifestation with pathological biopsy as well as corresponding immunohistochemical tests.