Key Clinical MessageNeuromyelitis optica spectrum disorders can less commonly present with area postrema syndrome progressing to myelitis. Management involves intravenous glucocorticoids, plasma exchange, and preventive immunotherapy.AbstractNeuromyelitis optica spectrum disorders can less commonly present with area postrema syndrome progressing to myelitis. The majority of patients have positive AQP4‐Ab. Diagnosis is based on clinical and imaging findings. These patients can be treated with intravenous glucocorticoids, plasma exchange, and preventive immunotherapy.