Brucellosis is a common zoonosis in many parts of the world, including Mediterranean and Middle Eastern countries. The disease is primarily related to occupations at risk, such as veterinarians, farmers, laboratory technicians, abattoir workers, and others working with animals and their products. Neurologic complications of brucellosis are quite rare, ranging from 1.7 to 10% of those infected. To date, no cases of neurobrucellosis with hydrocephalus have been reported. A 38-year-old right-handed farmer complained of headaches, nausea, vomiting, gait disturbance, and sweating for 2 days. He also complained of bilateral hearing loss of 4 months duration. On neurologic examination, dysmmetry, dysdiadochokinesis, ataxia on the left, and bilateral sensorineural hearing loss existed. On cranial MRI, a communicating hydrocephalus was noted. Because the patient consumed fresh sheep cheese and was a farmer, brucellosis was considered in the differential diagnosis. Brucella agglutination was positive with a 1/320 titer in the blood and a 1/80 titer in the cerebrospinal fluid. Ceftriaxone, doxycycline, and rifampicin were administered and by the fourth week of treatment, the ataxia was markedly improved, and the patient was able to walk without support. His cranial MRI demonstrated a total regression of the hydrocephalus. As a result, we suggest that neurobrucellosis should be considered in patients with hydrocephalus, especially if they live in an endemic area for brucellosis, even in the absence of other systemic signs. © 2008 Elsevier B.V. All rights reserved.