Achieving a quick and correct diagnosis of neuromyelitis optica spectrum disorder (NMOSD) remains challenging, even after the discovery of disease-specific autoantibodies to aquaporin-4. Salient symptoms of NMOSD are non-specific, and some clinicians have confused the condition with multiple sclerosis or other disorders. Until recently, treatments to manage acute attacks and prevent future episodes were limited to off-label options like azathioprine, mycophenolate, and rituximab. However, within the last 2 years, the therapeutic landscape has widened significantly with FDA approval of eculizumab, inebilizumab, and satralizumab. To improve patient outcomes, neurologists would benefit from continuing education that keeps them up to date on treatment advances and best practices for making a timely, accurate NMOSD diagnosis.