This retrospective study analyzes clinical features and therapeutic outcome in 26 immunocompetent patients with primary central nervous system lymphoma (PCNSL). Most patients presented with personality changes. PCNSL lesions were mainly iso- or hyperdense, enhancing lesions on CT scan, hypointensive on T1-, and hyperintensive on T2-weighted MRI. Multiple lesions were found in about 60% of patients. Nine of 11 patients receiving radiotherapy alone showed complete remission (CR). Median survival time after diagnosis (MST) was 13 months. Seven patients received intravenous and intrathecal methotrexate, radiotherapy, and postirradiation intravenous cytarabine. Six of these patients had CR and 5 patients are alive in CR after a median follow-up of 12 months. Five patients received various other radiochemotherapy regimens (MST 6 months), and 3 patients died before receiving any radio- or chemotherapy. Our preliminary treatment results show a tendency to improved survival with radiochemotherapy. This is consistent with pertinent data from the literature which favors radiochemotherapy for patients with PCNSL.