Neuroschistosomiasis caused by Schistosoma mansoni (Sm) is a rare and severe condition potentially leading to permanent neurological deficit. An 18-year-old Brazilian female was admitted due to a severe conus medullaris and cauda equina syndrome. MRI of thoracic/lumbar spine showed an expanded conus medullaris with patchy gadolinium-enhancement, needle electromyography revealed acute bilateral radiculopathy (L5-S1-S2), cerebrospinal fluid (CSF) showed lymphocytosis and increased proteins and lesion' surgical biopsy documented a lymphocyte infiltrate. Immunodiagnosis with cercariae hullen reaction using Sm cercariae in CSF and serum and immunoelectrodiffusion for circulating antigens detection using anti-Sm antibodies were positive. No schistosoma parasites were found. The patient was treated with praziquantel and corticotherapy for 6 months. At 1 month, partial clinical improvement was noticed, and MRI showed a normal size conus medullaris. At 6 months, there was complete clinical recovery. This case shows that a severe neurological deficit by Sm may have a clinical full recovery after treatment.