Background: Behcet’s disease (BD) is a multisystem autoimmune relapsing vasculitis with almost unknown etiology, which involves both large and small vessels. Involvement of the central nervous system (CNS) is rare condition devided in two main sub-types. First, the parenchymal type is caused by development of an immunemediated meningoencephalitis, which predominantly involves the brainstem and diencephalon region. The other type - non-parenchymal type is a consequence of thrombosis within the dural venous sinuses. Peripheral nervous system generally preserved or involved in very rare cases. Objective: To present a rare case of Neuro-Behcet Disease (NBD) and to make a thorough review on the clinical presentation, classification and neuroimaging findings. Methods: We searched the Pub Med electronic database with the keywords - Behcet Disease, Neuro-Behcet Disease and Neuroimaging. The literature search was conducted for the period from January 2000 to March 2020. Results: A total of 35 papers met the inclusion criteria and were included in the review. The review shows that NBD remains a difficult diagnosis to establish as other diseases and conditions may have similar presentation. Therefore we present the most common clinical signs and symptoms, classification, and diagnostic criteria with accent on the neuro-imaging findings. Conclusions: The diagnosis of NBD is based on the clinical presentation and the typical lesions in brain magnetic resonance imaging (MRI). The development and disappearance of lesions at MRI may correlate with the course of clinical neurologic deficits. Differential diagnosis of NBD should be considered in cerebrovascular disease, brain tumors, and demyelinating processes.