Neurosarcoidosis is known as the great mimicker and may appear similar to lymphoma, multiple sclerosis, and other diseases affecting the nervous system. Although definitive diagnosis requires histologic confirmation of the affected neural tissue, characteristic clinical manifestations, gadolinium-enhanced MRI patterns and specific cerebrospinal fluid findings can help support the diagnosis in the absence of neural biopsy. An understanding of the common clinical presentations and diagnostic findings is central to the evaluation and management of neurosarcoidosis.