Abstract
Malignant peripheral nerve sheath tumor (MPNST) is an uncommon type of sarcoma that arises from a peripheral nerve or any tissue with nerve sheath differentiation. It does not have any specific symptoms and shows great variability in clinical and radiological findings. It is usually associated with neurofibromatosis type 1 (NF1). Malignant peripheral nerve sheath tumors mostly originate from soft tissue organs and rarely originate from the small bowel. Less than 20 cases of intestinal MPNSTs are recorded worldwide. Therefore, we present this unique case of a 78-year-old woman, with no history of NF1, who presented with abdominal pain and vomiting and was diagnosed postoperatively with MPNST in the small bowel. Prompted by this case, we reviewed the literature for cases of intestinal MPNSTs.