Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune central nervous system disease with inflammatory events recurring in the optic nerves and spinal cord. Neuromyelitis optica (NMO) is rare in children, and pediatric patients typically present with optic neuritis following infections. Lack of response to pulse-dose intravenous corticosteroids or recurrent relapse episodes warrant therapies such as plasma exchange, intravenous immunoglobulin (IVIG), and rituximab. In adult studies, tocilizumab has been used and found to reduce NMO relapse; however, limitations exist in the pediatric population due to the lack of studies and formal treatment guidelines. Gastrointestinal perforation (GIP) is a rare, life-threatening complication identified in clinical trials during tocilizumab therapy. We report a case of a fatal, infected GIP following a single dose of tocilizumab for refractory NMO in a 15-year-old male. This case highlights the need for more outcomes data when using tocilizumab to treat pediatric NMO. Additionally, an assessment of patient-specific risk factors should be outlined to better direct safe therapy and to minimize negative adverse outcomes.