Sarcoidosis is a systemic inflammatory disorder characterized by non-caseating granulomas with variable involvement of the lungs, central nervous system (CNS), skin, liver, bone, and eye. Neurosarcoidosis is similarly heterogeneous with diverse anatomical manifestations and response to therapy. The diagnosis of neurosarcoidosis is established by the clinical syndrome, imaging and histopathological findings, and exclusion of other causes. In this narrative review, we summarize recent advances in the diagnosis and treatment of neurosarcoidosis.