Schistosomal myelopathy (SM) is a rare neurological manifestation of schistosomiasis affecting mainly people in endemic areas, with symptoms such as paresthesias, back pain, paraplegia or urinary disorders. The article describes the case of a 27-year-old Mozambican man with chronic back pain and progressive leg movement disorder; MRI showed inflammation of the spinal cord from T12 to L4 and Schistosoma eggs were found in the cerebrospinal fluid. A systematic review of the literature identified 58 cases of MS with a median age of 25 years (range 2–65), mostly in men (79%) with a subacute or chronic course. Lesions occurred mainly in the thoracolumbar region or conus medullaris, Schistosoma eggs were detected in 31% of cases and systemic manifestations such as bladder involvement in 64%. All patients received anthelmintic treatment and corticosteroids. At the end of the follow-up, 90% of patients could walk without assistance, 10% remained functionally dependent (modified Rankin scale ≥ 3). Early diagnosis is key to minimizing damage.