Neuromyelitis optica (Devic’s disease) is a chronic autoimmune disease associated with the production of anti-bodies to aquaporin-4 (AQP4). Area postrema lesions is the third, after optic neuritis and myelitis, syndrome of opticomyelitis-related disorders. Clinical symptoms of this disorder include intractable nausea, vomiting and hiccups. In many cases, area postrema syndrome manifests as the first clinical symptom of a neuromyelitis optica spectrum disorder that hampers the diagnosis. The authors present a case report of a female patient with area postrema lesions developed several months before the first disabling attack of myelitis.