Neurosyphilis literally means syphilitic infection of the central nervous system, but it is often referred to incorrectly as "tertiary syphilis." Neurosyphilis can occur at any time in the course of syphilis, even in the earliest, primary, stage. Early forms of neurosyphilis primarily affect the meninges, cerebrospinal fluid, and cerebral or spinal cord vasculature. Late forms of neurosyphilis primarily affect the brain and spinal cord parenchyma. Uveitis and hearing loss related to syphilis are most common in early syphilis and may be accompanied by early neurosyphilis. The treatment for syphilis-related eye disease and hearing loss is the same as the treatment for neurosyphilis. Neurosyphilis is more commonly seen in patients infected with HIV, and much of the recent literature pertains to this risk group. This article provides a critical review of recent literature on the diagnosis, clinical findings, risk factors, and management of neurosyphilis.