ABSTRACT BACKGROUND: Neuromyelitis Optica spectrum disorder is an inflammatory disorder affecting the central nervous system), most commonly attacking the spinal cord or optic nerves. Limited cases of neuromyelitis optica have been reported in east Africa. Based on my review, if published, this would be the second published case of Neuromyelitis Optica spectrum disorder and the first published case of seropositive Neuromyelitis Optica spectrum disorder reported from Ethiopia.  It signifies the need to have a high index of suspicion to promptly identify and properly treat these patients.CASE PRESENTATION: I am reporting a 32 years old female patient from Addis Ababa, Ethiopia, who presented with recurrent lower limb weakness and impairment of right eye vision of two-year duration. She was diagnosed based on Neuromyelitis Optica spectrum disorder diagnostic criteria, by having transverse myelitis, optic neuritis, confirmed by MRI imaging and high level of aquaporin-4-antibodies. Symptoms improved after providing five days of Methylprednisolone followed by low doses of corticosteroids and Azathioprine. The patient is now fully functional except for the right eye vision impairment.CONCLUSION: The patient described here signifies a classic manifestation of Neuromylitis Optica disorder with aquaporin-4-IgG occurring in Ethiopian woman. This case highlights the existence of Devic’s disease within our setting and the need to properly diagnose this condition even in a resource-limited setting to avert disability.KEYWORDS: Neuromyelitis Optica, Aquaporin-4 antibodies, case report, Ethiopia