Neuromyelitis optica spectrum disorder (NMOSD) is a rare disease associated with the development of antibodies to the central nervous system. Long segment spinal cord demyelination and inflammation (myelitis), severe optic nerve involvement and/or persistent vomiting and hiccups (area postrema syndrome) are the clinical forms that are important for the diagnosis of the disease. 1 Approximately 20-30% of the patients with NMOSD have a coexisting organ-specific or systemic autoimmune disease. 2 It is critical to know that there may be another autoimmune disease or diseases accompanying NMOSD and to consider it in the differential diagnosis. Here, we present a case of NMOSD with concurrent multiple autoimmune diseases, we aimed to highlight unique clinical features and the challenges encountered in diagnostic and treatment management.