Diagnostic criteria for neuromyelitis optica (NMO) state that there should be no active disease outside the optic nerves and spinal cord. However, several cases have been described with symptomatic brain involvement. We describe an autopsy case of a patient with NMO and symptomatic involvement of the brain. The histopathology of the brain lesions is typical for NMO, with extensive macrophage infiltration, including perivascular accumulation of large numbers of eosinophils. This is the first case that clearly shows that in NMO, the histopathology of the brain lesions is identical to that of the lesions in the optic nerves and spinal cord.