Malignant schwannomas are rare. These malignomas are primary nerve sheath tumors that usually arise from a peripheral nerve. They appear most frequently in the lower extremities, including the hips and buttocks (34.8 %), the upper extremities (23.4 %), and in the trunk (17 %). To the best of our knowledge, this is the first reported case of a malignant schwannoma of the rectum. We describe the rare disease with reference to the literature.