A 60-year-old man presented with progressive and unique neurological symptoms. Investigations identified an isolated cerebellar lesion. This lesion fulfilled the histological criteria for lymphomatoid granulomatosis, and in situ hybridization and deoxyribonucleic acid (DNA) dot blot techniques revealed significant amounts of Epstein-Barr virus DNA within the tumor cells. The patient underwent cranial radiation therapy, and 16 months after the initial presentation the lesion evolved into a malignant lymphoma. He subsequently died secondary to subdural empyema, bacterial meningitis, and bronchopneumonia. The unique clinical and etiological aspects of this case are addressed.