Objective: Neuromyelitis optica (NMO), also known as Devic's disease, is a rare inflammatory condition of the central nervous system impacting the optic nerve and spinal cord, often resulting in visual changes and mobility impairment. Recent literature suggests that NMO may be associated with cognitive problems, similar to those associated with multiple sclerosis, in adults. However, no studies have examined neuropsychological and academic outcomes in pediatric NMO. The objective of this study is to describe neuropsychological and academic functioning in a pediatric NMO sample. Method Thirteen participants, ages 6 to 19 years (mean = 12.6) diagnosed with NMO, were administered a neuropsychological screening battery, including standardized rating forms of behavioral, emotional, and executive functioning, during a specialty clinic visit. Results One-sample t-tests revealed statistically significant differences from the mean (p ≤ .05) for visual-motor integration and bilateral fine-motor skills. Ratings of initiation, working memory, planning/organization, and somatization were also significantly different from the mean (p < .05). Furthermore, approximately one-third of the sample showed impairment with memory, auditory attention, and verbal fluency. Fifty percent showed impairment in working memory. Results of standardized behavioral ratings revealed that approximately one-third had elevations in the areas of attention, emotional control, depression, and withdrawal. Additionally, 46% had elevations in the area of anxiety. Regarding academics, 33% were experiencing school problems. Conclusion Results suggest that pediatric NMO patients are at risk for cognitive and motor impairment, as well as difficulties related to emotional functioning and academics. Novel findings from this study support the need for a multidisciplinary approach in the care of this population.