Neurosarcoidosis is frequently on the differential diagnosis for neurohospitalists. The diagnosis can be challenging due to the wide variety of clinical presentations as well as the limitations of noninvasive diagnostic testing. This article briefly touches on systemic features that may herald suspicion of this disorder and then expands in depth on the neurological clinical presentations. Common patterns of neurological presentations are reviewed and unusual presentations are also included. A discussion of noninvasive testing is undertaken, exploring dilemmas that may be encountered with sensitivity and specificity. Drawing from a broad range of clinical clues and diagnostic data, a systematic approach of pursuing a potential tissue diagnosis is then highlighted. Correctly diagnosing neurosarcoidosis is critical, as treatment with appropriate immunosuppression protocols can then be initiated. Additionally, treatment of refractory disease, the trend toward exploring targeted immunomodulation options, and other therapeutic issues are discussed.