Cerebral angiotropic large cell lymphoma (neoplastic angioendotheliosis) is a rare disease with a particular propensity to affect the central nervous system by vascular occlusion. Because the disease is rare and there are no specific diagnostic procedures apart from cerebral biopsy, it is difficult to diagnose in life. Accordingly, chemotherapy or radiotherapy has only rarely been attempted and their effectiveness is uncertain. We established the diagnosis in a 62-year-old patient by cerebral biopsy and observed remission following institution of combination chemotherapy. Unfortunately, neurologic deterioration recurred during maintenance chemotherapy. We identified 30 patients in the literature who initially presented with definite central nervous system manifestations and whose clinical conditions were described sufficiently enough for comparison with our case. Eleven patients had the diagnosis made in life, but only 5 received chemotherapy other than corticosteroid hormones. Our patient's survival for 16 months exceeded that in the majority of the 30 reported cases. Intense anti-lymphoma chemotherapy, and possibly radiotherapy, may be beneficial and should be studied in this otherwise rapidly fatal disease. It seems certain that early diagnosis is essential if therapeutic intervention is to be successful.