Schistosomiasis is a parasitic disease caused by blood flukes of the genus Schistosoma. Currently 200 million people worldwide are infected. Neurological manifestations are a result of the inflammatory response of the host to egg deposition in the brain and spinal cord and is usually seen in patients with recent infection with no evidence of systemic illness. Cerebral and cerebellar disease can result in headache, seizure, and increased intracranial pressure. Cerebral schistosomiasis is more common in Schistosoma japonicum, but increasing cases due to Schistosoma mansoni are being reported in the literature. Other complications of cerebral schistosomiasis include delirium, loss of consciousness, visual field impairment, focal motor deficits, and ataxia. Myelopathy is the most common neurological manifestation of Schistosoma mansoni and the conus medullaris and cauda equine are the most common sites of involvement. Severe disease can result in flaccid paraplegia with areflexia, sphincter dysfunction, and sensory disturbance. Early recognition and prompt treatment are essential when physicians are faced with schistosomiasis involving the central nervous system. Schistosomicidal drugs, such as praziquantel, steroids and surgery, are the mainstay of therapy for this severe form of schistosomiasis.