Neuromyelitis Optica Spectrum Disorder (NMOSD) is an autoimmune, demyelinating disorder of CNS which causes recurrent inflammatory events involving spinal cord, optic nerve and also brainstem. These areas are abundant in AQP4 water channels, antibodies against these channels (AQP4-IgG) are highly specific for NMOSD. Majority of the patients presents with the classical symptoms of transverse myelitis and optic neuritis while rarely may present with brainstem symptoms only like vomiting and hiccups. Here, we are reporting a case of a four years old girl, who presented with complains of recurrent episodes of vomiting and weight loss for 3 months, without any other associated symptoms. She was thoroughly worked up for gastrointestinal causes of vomiting and nothing was conclusive. With this clinical picture, child was diagnosed to have NMOSD on the basis of new NMOSD diagnostic criteria. She responded well to the therapy and she is now under regular follow up.