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We present the case of a 54-year-old patient treated with cemiplimab, an immune checkpoint inhibitor (ICI), for multiple basal cell carcinomas in the context of Gorlin Goltz syndrome. Gorlin Goltz syndrome is an autosomal dominant multisystem disorder characterized, among other features, by multiple early-onset basal cell carcinomas (BCCs). After receiving Cemiplimab, she developed aquaporin-4 antibody (AQP4-Ab) positive neuromyelitis optica spectrum disorder (NMOSD). While several case reports have documented NMOSD induced by other ICIs, this is the first case associated with cemiplimab. Although guidelines exist for the acute treatment of a first relapse of ICI-induced NMOSD, long-term management to prevent new relapses remains challenging. We believe that these patients require maintenance therapy to prevent future relapses and propose rituximab or tocilizumab as suitable options. •Immune checkpoint inhibitors (ICIs) work by activating the immune system to target cancer cells. However, this activation can sometimes trigger immune-related adverse events (irAEs).•Neuromyelitis optica spectrum disorder (NMOSD) can occur as an irAE induced by the immune checkpoint inhibitor (ICI) cemiplimab.•We propose long-term immunosuppression in patients with ICI-induced NMOSD to prevent further relapses and potential disability.•Due to their mechanism of action and well-established efficacy in both NMOSD and irAEs, tocilizumab and rituximab appear to be promising options for long-term immunosuppression in ICI-NMOSD.