OBJECTIVE Blood fluke infection or schistosomiasis affects up to 300 million people worldwide. Although infection of the hepatic and urogenital systems commonly occurs, central nervous system involvement is rare. When presenting in the spinal cord, schistosomiasis can be difficult to diagnose because it can present as mass lesion or transverse myelitis. We describe a patient with lumbar intramedullary spinal schistosomiasis who presented to a tertiary medical center in the United States and discuss the diagnosis and treatment of this rare disease. METHODS A 25-year-old Brazilian man presented with progressive, subacute, bilateral lower extremity motor and sensory deficits and disturbances in bladder function. Magnetic resonance imaging revealed a diffusely enhancing mass expanding the region of the conus medullaris. METHODS Laboratory investigation disclosed serum and cerebrospinal fluid eosinophilia. Ultimately, cerebrospinal fluid serology demonstrated the presence of Schistosoma mansoni. The patient was treated with praziquantel and steroids and made a marked recovery. CONCLUSIONS The clinical and radiographic manifestations of spinal schistosomiasis can mimic those of intra-axial spinal tumors and transverse myelitis. To avoid unnecessary surgery or delay in treatment, the clinician must have knowledge of this type of presentation. The increasing volume of international travel and high prevalence of the disease worldwide increases the possibility that the practicing neurosurgeon in the United States may encounter this rare but treatable disease.