An unusual case of a 34‑year‑old woman with a malignant peripheral nerve sheath tumor (MPNST) associated with neurofibromatosis type 1 (NF1), arising from plexiform neurofibroma of the mesentery is presented here. She presented with complaints of abdominal pain of 10 days duration. Imaging revealed the presence of multiple nodules along with a large mass in the mesentery of the small bowel. Small bowel resection along with the mesenteric mass was done and histopathologic examination showed MPNST arising from a plexiform neurofibroma of the mesentery. A review of the literature showed that till date only three cases of MPNST arising from neurofibroma of the mesentery in NF1 patients have been reported out of which one was from a plexiform neurofibroma.