Neuromyelitis optica spectrum is order (NMOSD) is a rare auto-antibody mediated disease of the central nervous system involving the central nervous system. The symptoms should alert the clinician to the diagnosis. Prompt diagnosis in the emergency department plays a crucial role as delay in diagnosis and initiation of management will lead to increased chances of morbidity and mortality. Approximately 80% of NMO patients have auto-antibodies against aquaporin-4, a water channel expressed on astrocytes. In this case report, we discuss a patient with NMO disease, the diagnosis and its management.