A case of jejunal-ileal metastasis from a left leg malignant schwannoma in a 73 year old woman is described. The patient was affected by progressive anemization without signs of bleeding. A colonoscopy performed during hospitalization revealed an ulcerative lesion at the ileo-colonic junction which at histologic examination was suspected for sarcomatous proliferation arising in peripheral nerve tissue. At operation multiple ileal resections of numerous polypoid formations were performed. Histological examination of polypoid masses revealed the presence of malignant proliferation of spindle cells consistent with a malignant schwannoma. Differential diagnoses are discussed with a review of the literature.