Tick-borne diseases are prevalent throughout the world and present a diagnostic challenge owing to their nonspecific clinical symptoms. Many tick-borne diseases involve the central and peripheral nervous systems. Early diagnosis or at least suspicion of a tick-borne cause is necessary to institute early empiric treatment. After a brief review of tick biology, we present the most common tick-borne diseases. A brief discussion of epidemiology, the transmission route, and pathogenesis is followed by a discussion of the clinical manifestations, diagnosis and treatment options when available. The review emphasizes the infectious causes with a significant neurological manifestation.