Brucellosis is a multisystem disease with diverse clinical presentations, and involvement of the nervous system is considered to be rare in childhood. Five children with meningitis (n = 2), meningoencephalitis (n = 1), meningomyelitis (n = 1), or cerebellar ataxia (n = 1) are described, all of whom had a history of exposure to a possible source of brucellosis. Examination of cerebrospinal fluid (CSF) revealed lymphocytic pleocytosis in 4 patients, high protein concentration in 5 and low glucose concentration in 3. Reciprocal brucella agglutination titers were significantly elevated in serum (greater than or equal to 160) and in CSF (greater than or equal to 80) of all patients. Brucella melitensis was isolated both from blood and CSF in one patient, from blood only in 2, and from bone marrow only in another one. All patients were treated successfully by a three-drug combination of streptomycin (4 patients) or doxycycline (one patient) with trimethoprim-sulfamethoxazole and rifampin, and in one patient dexamethasone was also added. In endemic areas, neurobrucellosis should be suspected in the evaluation of patients with unexplained neurologic symptoms.