A 71-year-old man presented with diplopia and periorbital pain. The clinical examination showed left-sided incomplete external oculomotor nerve palsy. The remainder of the neurological and ophthalmological examination was unremarkable except for slight polyneuropathy. He was on medication treating diabetes and arterial hypertension. Two months earlier a diffuse large cell B-lymphoma was diagnosed based on histology of a pleura biopsy. He was treated with three cycles of chemotherapy according to the R-CHOP scheme (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone). Cerebral magnetic resonance imaging (MRI) demonstrated no abnormal finding (Fig. 1a). The lumbar puncture revealed pleocytosis (600 cells pro µl; normal <4), elevated protein (658 mg/dl; normal <450), elevated lactate (6.7 mmol/l; normal <2.2) and diminished glucose (35 mg/dl compared to serum 159 mg/dl). Antibody specificity indices of Borrelia burgdorferi, varicella virus, and herpes virus were within normal limits. By PCR no DNA of varicella zoster virus and herpes virus were detected. Cytopathological examination of the CSF showed a marked increase of the number of cells. The cells were relatively monomorphic and revealed blastic features compatible with diagnosis of meningeosis lymphomatosa (Fig. 1b, c). A high dose systemic chemotherapy was initiated.