Neuroschistosomiasis, the infection of the CNS by Schistosoma spp., is a neglected and under-recognized complication of schistosomiasis. Cerebral and spinal neuroschistosomiasis can provoke severe disability. Neurological symptoms occur as a consequence of the immune reaction around the eggs deposited in the CNS. Cerebral neuroschistosomiasis may present with altered sensorium, headache, seizures and focal neurological deficit. Pseudotumoral and cerebellar neuroschistosomiasis may provoke intracranial hypertension and hydrocephalus. Brain-enhancing lesions with associated mass effect can be observed on MRI. Transverse myelitis and myeloradiculopathy affecting the conus medullaris and/or cauda equina are the most common spinal cord syndromes. Transverse myelitis can present as flaccid arreflexic paraplegia with sensory level and sphincter dysfunction. Praziquantel and corticoids have been successfully used to treat neuroschistosomiasis. Ventricle-peritoneal shunt may be necessary to treat hydrocephalus associated with tumor-like brain and/or cerebellar schistosomiasis.