The paper reports a rare case of malignant ileal schwannoma. A review of the most recent literature confirms the diagnostic and therapeutic procedures used by the authors. Minimal resection or necessary resection in relation to the site and local situation are the only real therapy apart from palliative surgery. It is vital to identify any possible polycentric manifestation since schwannoma is often associated with Recklinghausen's disease. Radiotherapy and chemotherapy are completely ineffective. Responses were obtained in around 30% of cases in the most common trials; the gastrointestinal localization also appears to be even less responsive. The most active drugs are decarbazine, doxorubicin and ifosfamide, associated with CIVADIC or MAID polychemotherapy protocols. It is often difficult to diagnose malignancy; the overall survival rate (taking grading into account) is 7-10% after 10 years and 50% after 5 years for those forms with the lowest degree of malignancy. Antoni classified schwannoma into: type A with a solid component, and type B with a microcystic component. Treatment and prognosis are identical.