OBJECTIVE The authors present a retrospective analysis of 22 patients with primary central nervous system lymphoma(PCNSL) in order to provide a reasonable basis for the diagnosis and treatment of the disease. METHODS This report involves a clinicopathological study of 22 patients with histologically proven PCNSL, all diagnosed between January 1993 and May 2000. Univariate and multivariate regression analysis are used to determine prognostic factors significantly associated with an unfavorable or favorable impact on survival. RESULTS The cohort included 11 men and 11 women whose median age at diagnosis was 49.5 years. At the end point of follow-up, 11 died. The median survival time for the patients in study was 14.5 months. With univariate and multivariate regression analysis, prognostic factors significantly associated with survival included intrathecal injection(P = 0.005) and local/diffuse neurological deficit (P = 0.031). CONCLUSIONS There continues to be a significantly increasing incidence of PCNSL. This survey throws light on the clinical and prognostic features of this uncommon disease. Through univariate and multivariate regression analysis the authors highly recommend a therapeutic regime including surgery, intrathecal injection and chemotherapy, especially those drugs capable of passing blood-brain barrier, for example high dose MTX.