A 30-year-old patient with a relapsing primary central nervous system lymphoma (PCNSL), was successfully treated with salvage chemotherapy and high-dose therapy including drugs that cross the blood-brain barrier followed by ABMT. Cerebrospinal irradiation was administered after hematological recovery. Six years after transplantation the patient is alive without evidence of recurrent disease with a good neuropsychological status. This result could justify further studies of aggressive management of PCNSL in relapse.