The involvement of the central nervous system (CNS) by Schistosoma mansoni may or may not cause clinical manifestations. When symptomatic, neuroschistosomiasis mansoni (NSM) is one of the most severe presentations of this infection. The neurological manifestations are due to numerous granulomas grouped in confined areas of the spinal cord or the brain. Considering the symptomatic form, myelopathy is far more frequent than the cerebral disease. Spinal cord NSM presents as a low cord syndrome of acute/subacute progression usually associated with involvement of the cauda esquina roots. Lower limbs pain, weakness and sensory disturbance, and autonomic dysfunctions, particularly bladder dysfunction, are often present. Cerebrospinal fluid (CSF) examination generally shows an inflammatory pattern with or without eosinophils and/or IgG against schistosomal antigens. Magnetic resonance imaging (MRI) demonstrates signs of inflammatory myelopathy. Cerebral NSM presents as a slow-expanding intracranial tumor-like lesion. Its clinical manifestations are variable and depend on the increased intracranial pressure and on the site of the lesion. The diagnosis of spinal cord NSM is based on clinical evidence whereas the cerebral disease is usually diagnosed by biopsy of the nervous tissue. There is no consensus on the treatment of NSM. We discuss the literature data on this topic, and suggest a therapeutic approach based on our experience with 69 spinal cord NSM patients who have been followed up by a long period of time. Outcome is largely dependent on early treatment, particularly in the medullar disorder, and is better in cerebral NSM.