Inherited metabolic diseases represent a heterogeneous group of rare and sometimes treatable conditions that can mimic central nervous system (CNS) inflammatory or demyelinating disorders such as multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), or myelin oligodendrocyte glycoprotein antibody disease (MOGAD). This chapter provides a comprehensive review of inherited metabolic disorders with neurological involvement, including biotinidase deficiency, cerebrotendinous xanthomatosis, Fabry disease, remethylation disorders (e.g.,cobalamin metabolism defects), hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome, lysosomal and peroxisomal storage diseases (e.g., adrenoleukodystrophy, Krabbe disease, metachromatic leukodystrophy), mitochondrial disorders, and Wilson‘s disease. For each disorder, the chapter discusses underlying genetic and biochemical abnormalities, typical and atypical clinical presentations, neuroimaging findings, and laboratory features, including characteristic metabolic profiles. Imaging patterns such as longitudinally extensive spinal cord lesions or white matter hyperintensities may resemble inflammatory demyelinating diseases and thus lead to misdiagnosis. Cerebrospinal fluid (CSF) findings are often normal, but restricted oligoclonal bands (OCB) may be present in some metabolic disorders. Diagnostic strategies involve enzyme activity testing, genetic sequencing, and specific metabolic assays (e.g., plasma cholestanol, homocysteine, very long-chain fatty acids). Importantly, many of these disorders are amenable to specific treatments, including vitamin or cofactor supplementation, dietary modifications, enzyme replacement therapy, or hematopoietic stem cell transplantation. However, delayed recognition frequently leads to irreversible neurological damage. This chapter highlights diagnostic clues, imaging red flags, and key laboratory findings that can help clinicians distinguish inherited metabolic disorders from autoimmune CNS diseases and emphasizes the importance of early diagnosis and targeted therapy.