Brucellosis is a multi-system infectious disease that presents with various clinical manifestations and complications. Neurobrucellosis is an uncommon but serious presentation of brucellosis that can be seen in all stages of the disease. Neurobrucellosis is a focal complication of brucellosis affecting both central and peripheral nervous system presenting varieties of signs and symptoms. The most reported manifestations are meningitis and meningoencephalitis. It is a rare presentation of brucellosis. The estimated mean incidence of neurobrucellosis is 1.7%-10%. The incidence is equal in males and females. Initial clinical manifestations consisted of meningoencephalitis, acute and subacute meningitis, intracranial hypertension, polyradiculoneuritis, cerebral and subarachnoid hemorrhage, transverse myelitis, lumbar epidural abscess with root involvement, and cranial nerve involvement. Other rare manifestation includes pseudotumor cerebri, intracranial granuloma, sagittal sinus thrombosis, spinal arachnoiditis, and intracranial vasculitis. High index of suspicion, especially in endemic areas is essential to prevent morbidity from this disease. Clinical suspicion and accurate evaluation of a patient's history is the most important clue in diagnosis and treatment. Neurobrucellosis can be diagnosed by isolation of microorganism from the CSF or detection of antibodies in the CSF. The CSF pattern in neurobrucellosis can be helpful for diagnosis; lymphocytic pleocytosis, increased protein, and decreased glucose levels in the CSF are in favor of neurobrucellosis. Imaging modalities, including CT scan or magnetic resonance imaging, may reveal information for diagnosis. Many laboratory procedures are usually employed in the diagnosis of neurobrucellosis. Even though the culture method is the gold standard, growth rate is low and time consuming. Coombs' test should be performed in both the CSF and serum. Different regimens are usually used based on ceftriaxone, doxycycline, cotrimoxasole, streptomycin, and rifampicin. Treatment with intravenous ceftriaxone and oral rifampicin, doxycycline, and trimethoprim-sulfamethoxazole resulted in a good clinical response. Patients with severe and persistent headache and other neurologic symptoms and signs should be considered for neurobrucellosis in endemic regions. Early diagnosis and treatment of neurobrucellosis will be helpful in decreasing the sequelae of this complication.