目的　报道 6例原发性中枢神经系统淋巴瘤 (PCNSL)患者的临床资料 ,探讨这一少见病的临床特点、诊断及治疗。方法　对 6例 PCNSL患者的临床、影像、病理资料及治疗等进行全面分析。结果　免疫状态改变或病毒感染可能与发病有关。此病男性多见 ,中年为主 ,急性或亚急性起病 ,病程较短 ,进展较快 ,症状、体征多样 ,CT呈等密度或高密度 ,磁共振 (MRI)呈长 T1长 T2 ,周围有水肿 ,有结节状或环状增强 ,组织学符合弥漫型B细胞型非霍奇金淋巴瘤 (NHL) ,对糖皮质激素有一定治疗反应。其中 3例生存期分别已达 9、3 3、3 9个月 ,2例失访 ,1例死亡。结论　结合 PCNSL临床表现、影像及病理学特点可对此病作出诊断 ,早期治疗有益预后 Objective To report the clinical data of 6 patients with primary central nervous system lymphoma (PCNSL) and discuss the clinical features, diagnosis and treatment of this rare disease. Methods The clinical, imaging, pathological data and treatment of 6 patients with PCNSL were analyzed comprehensively. Results of immune status changes or viral infection may be related to the incidence. This disease is more common in men, middle-aged, acute or subacute onset, shorter duration, faster progress, various symptoms and signs, CT showed equal density or high density, magnetic resonance (MRI) showed long T1 long T2, Around the edema, nodular or annular enhancement, histology in line with diffuse B-cell non-Hodgkin’s lymphoma (NHL), glucocorticoid have a certain therapeutic response. Three of them survived 9,3, 3 and 9 months respectively, 2 were lost and 1 died. Conclusion PCNSL combined with clinical manifestations, imaging and pathological features can make a diagnosis of the disease, early treatment of beneficial prognosis