Behçet’s disease (BD) is an autoimmune vasculitis of unclear etiology characterized by a wide range of systemic manifestations. The nervous system is involved in approximately 10% of patients with BD (neuro-Behçet). Diagnosis of neuro-Behçet is challenging, especially in the case of BD onset with neurological manifestations, and is based on a combination of clinical, laboratory, and neuroimaging data. To verify the diagnosis of neuro-Behçet, a wide range of conditions with similar manifestations must be ruled out: multiple sclerosis, neuromyelitis optica spectrum disorder, neurosarcoidosis, and many rare autoimmune diseases (e.g., Cogan syndrome, Susac syndrome, Vogt-Koyanagi-Harada syndrome). This article presents a brief literature review highlighting the challenges of neuro-Behçet differential diagnosis and a clinical case of a 34-year-old male patient with a criteria-based diagnosis of BD with neurological manifestations.