A 34 year-old woman presented a subacute onset of right hemiparesis and right blurred vision. The head and spinal cord MRI revealed a longitudinally extensive transverse C5-D1 myelitis (LETM) and a posterior superior right optic nerve lesion, with peripheral irregular enhancement. No recent infections/systemic symptoms or other diseases were reported. General examination was normal. Neurological exam was characterized by moderate-to-severe ataxo-spastic gait, right weakness, left sensory loss and impaired vision in lower right eye field.