A 2-year-old Brazilian boy presented with bilateral leg weakness and constipation, followed by development of progressive paraparesis and bladder dysfunction. Neurological examination revealed flaccid paraparesis. Blood tests and CSF analysis showed eosinophilia. The MR examination revealed a spinal cord mass extending from T9 to L1 levels, with a heterogeneously-enhancing solid component and a cystic component. Stool tests for Schistosoma mansoni eggs were positive. The patient underwent surgery, the intramedullary mass was partially resected, and the diagnosis of spinal cord infection by Schistosoma mansoni was confirmed. After surgery, the patient was treated with praziquantel and oxamniquine. He was discharged with partial improvement of the lower extremity weakness and bowel/bladder function. The clinical and imaging features of spinal cord schistosomiasis are reviewed.