A young Puerto Rican male presented acutely ill with a neurogenic bladder, fecal incontinence and weakness of the lower extremities without sensory disturbances. Studies revealed Schistosoma mansoni involvement of the upper lumbar spinal cord. Histological examination of the involved portion of cord suggested the presence of an ectopic female adult worm in an adjacent spinal vein may have been responsible. The patient recovered with stibophen and corticosteroid therapy. A review of the pertinent literature indicates that biopsy-proven schistosomiasis of the spinal cord is rare. The present report is the third biopsy-proven case of S. mansoni of the spinal cord in continental United States.