We present a 32-year-old female patient with fulminant neuromyelitis optica. After the initial treatment with the monoclonal antibody rituximab failed, therapy with the anti-IL-6 receptor antagonist tocilizumab was initiated. The patient experienced a clinically relevant improvement from severe tetraparesis to low-grade paresis, which is still maintained. On MRI of the spinal cord an almost complete restitution of a predescribed extensive myelopathy accompanied this clinical improvement. Meanwhile clinical stability was achieved for over 1 year without any side effects of the ongoing treatment with tocilizumab.