Neurologic involvement in patients with familial Mediterranean fever is relatively uncommon, and rarely described in the literature. Although headache occurs frequently, meningitis and convulsions are rare. We describe the case of a 30-year-old man with attacks of meningitis. After colchicine therapy, no further recurrence of fever and meningitis were observed. These findings suggest that meningitis should be considered as an unusual manifestation of familial Mediterranean fever.