A 23-year-old male presented with acute headache, fever and neck pain associated with urinary retention. Initial exam showed nuchal rigidity, and a full neuroaxis MRI was unremarkable. He was empirically treated for meningitis. CSF revealed lymphocytic pleocytosis with negative extensive microbial studies. Course was complicated by paraplegia and acute encephalopathy requiring intubation. Repeat MRI showed diffuse cortical encephalitis, brainstem leptomeningeal enhancement, dotted cerebellar and ganglionic perivascular enhancement, and a non-enhancing cervical longitudinally extensive myelitis. Repeat CSF was positive for unmatched oliogoclonal bands and GFAP-IgG by cell-based assay. Body PET was negative for neoplasms. He was treated with pulse corticosteroids, plasmapheresis, and rituximab with subsequent mental status improvement and extubation. Subsequent MRI showed cortical improvement but persistence of the brainstem changes. Upon long-term follow-up, he was walking independently and had returned back to his normal baseline.