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Background Immunoglobulin G 4 (IgG4) related disease (RD) is a multisystem, immunologically mediated disease discovered within the last two decades. Within the nervous system, a broad range of both central and peripheral nervous system involvement has been reported. We aimed to systematically review the neurological manifestations of IgG4-RD.Aim To identify the clinical presentation, radiological findings, diagnostic methods, treatment and outcomes for neurological manifestations of IgG 4-RD.Methods We systematically reviewed the literature to identify all reports of neurological manifestations of IgG 4-RD. Data on neurological manifestations, non-neurological manifestations, clinical presentation, radiological findings, diagnostic methods, treatment modalities and outcomes were extracted.Results We identified 393 cases from 297 publications meeting the inclusion criteria. Hypertrophic pachymeningitis, IgG 4-related orbital disease and hypophysitis are the most common neurological manifestations of IgG4-RD. Diagnostic evaluation involves testing for concomitant non-neurological manifestations, an MRI with gadolinium contrast, measurement of serum IgG4 levels and a biopsy with specific staining for IgG4 positive plasma cells. Treatment with corticosteroids leads to favourable outcomes.Conclusions IgG 4-RD is an emerging neurological disease that can manifest in multiple ways within the nervous system. It is important to recognise as treatment is often successful.