Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory condition of the central nervous system caused by severe immune-mediated demyelination and axonal destruction, mainly affecting optic nerves and the spinal cord. We describe a 26-year-old Nepalese woman with recent onset of headache, nausea, vomiting and hiccups indicative of Area Postrema Syndrome (APS). The antibody test for aquaporin-4 was strongly positive. Brain magnetic resonance imaging (MRI) showed a bilateral hyperintense signal in the area postrema (AP). The patient started on methylprednisolone, and then azathioprine was added. However, the patient was readmitted because of tingling in her right upper extremity and sudden onset of tremors. An MRI scan showed an enlarged lesion in AP. Rituximab was started on top of the previous treatment, and a second dose was given after 2 weeks. The patient had been monitored regularly and symptom-free for 5 months. Hence, we emphasize the immediate need for a diagnostic approach for NMOSD management.