Object The authors performed a study to investigate the clinical manifestations, treatment strategies, and possible pathogenesis of conus medullaris schistosomiasis. Methods Six cases collected from the authors’ experience and four cases reported in the literature were studied retrospectively for clinical manifestations, treatment outcomes, and prognosis. All patients experienced progressive lower-extremity weakness and functional bowel and bladder impairment. Although the magnetic resonance (MR) imaging results suggested the presence of a conus medullaris tumor, schistosomiasis was diagnosed based on pathological results obtained in the 10 patients. The results of surgery followed by pyquiton and hormone treatment confirmed the diagnosis, and the patients’ prognoses were good. Conclusions This pathological entity is predominantly found in adults, and the clinical manifestations have no specificity, although the MR imaging may provide some clues. As a form of ectopic schistosomiasis, conus medullaris schistosomiasis deserves special consideration and further exploration. If an early diagnosis can be made and pyquiton and hormone therapy is given, surgery can be avoided and the prognosis will remain good.