Sarcoidosis with lesions in the central nervous system is a heterogeneous condition with diverse clinical presentations and various degrees of neurological disability. Although extremely rare among sarcoidosis cases, it is of relevance to routine clinical practice because it may be a differential diagnosis in brain tumors, meningitis, and myelopathy of unknown origin. Encephalic and myelopathic lesions of sarcoidosis are often resistant or only partially responsive to immunotherapies, including high-dose corticosteroids and immunosuppressants. This may lead to residual neurological disability in a certain number of cases. Several retrospective findings have recently shown superior therapeutic effects with infliximab. However, high-level evidence is warranted to confirm its efficacy to treat neurosarcoidosis.