A 61-year-old man with multiple subcutaneous, ileocecal and neurologic manifestations was reported. Histological examination of subcutaneous and ileocecal mass showed non-Hodgkin's lymphoma (diffuse medium cell type [LSG classification]), B-cell type. Headache, somnolence and incontinence of urine were considered owing to the CNS involvement by lymphoma cells. The cranial CT findings showed diffuse spread involving the lateral and third ventricle and hypothalamus with adjacent edema. Then he was treated by VEMP regimen and repeated intrathecal MTX injections. The neurologic remission with improvement of cranial CT findings was obtained and he has been free of the disease for 15 years.