Neuromyelitis optica (NMO) is a rare and severe inflammatory disease of the central nervous system (CNS), which constitutes up to 5% of pediatric aquired demyelinating diseases. The optic nerves and the spinal cord are the most affected sites. The discovery of an autoantibody called NMO-IgG, which targets aquaporin-4, the main water channel in the CNS, gave a new direction to understanding the underlying immunologic mechanisms. This specific biomarker also helps to distinguish the disease from other demyelinating disorders. Here, we review the clinical and paraclinical features, immunological properties and treatment options of the disease as reported in the literature.