原发性中枢神经系统淋巴瘤(PCNSL)是指病灶局限于中枢神经系统(包括脑实质、脊髓、颅神经、眼和软脑膜)而无全身播散证据的结外非霍奇金淋巴瘤(NHL)，是一种少见的儿童和青少年NHL亚型。先天性或获得性免疫缺陷患儿患此病风险更高。由于缺乏儿童前瞻性研究，现对近年儿童PCSNL的病例报道和成人前瞻性研究进行汇总，以帮助读者更好理解手术、全脑放疗和化疗在其治疗中的作用。“,”Primary central nervous system lymphoma (PCNSL) is a form of extranodal non-Hodgkin lymphoma (NHL) and it is typically confined to brain, spinal cord, cranial nerves, eyes, and meninges without evidence of system spread.It is a rare subtype of NHL in childhood and adolescence, with an increased risk among patients with immunodeficiency.In the absence of prospective pediatric clinical trials, data from recent pediatric series and adult prospective trials was summarized, so as to help audience better understand the role of surgery, whole brain radiation therapy, and chemotherapy in the treatment as well.