Neuromyelitis optica (NMO or Devic's syndrome) is a rare autoimmune disease, previously considered a multiple sclerosis variant. The most important laboratory and clinical features are optic myelitis and transverse myelitis, associated with neuromyelitis optica-IgG antibody (NMO-IgG) positivity. Subsequent to this immunological test being available, different groups have described the not-so-rare comorbidity of neuromyelitis optica with other systemic autoimmune diseases, systemic lupus erythematosus with secondary anti-phospholipid syndrome (APS) in particular. We describe a patient meeting both the classification criteria for primary APS and the new diagnostic criteria for neuromyelitis optica. It's important to diagnose NMO syndrome as both optic neuritis and transverse myelitis were also considered neurological complications of antiphospholipid syndrome. NMO-IgG is a new and fundamental test to decide if immunosuppressant therapy is warranted for such patients.