Neurofibromatosis type1 (NF1), also known as Von Recklinghausen’s disease with approximate occurrence of 1/3000 births, can be considered as a common autosomal dominant condition. Abdominal organs can be involved as neurofibroma and tumor growth in the liver, mesentery, retroperitoneum, stomach and bowel. Association of NF and gastrointestinal neoplasms occur in 2%-25% of patients. There is a 5%-10% lifetime risk of developing an NF1-associated malignant peripheral nerve sheath tumor (MPNST), usually arising within a pre-existing plexiform neurofibroma which metastasizes widely heraldinga poor outcome. We report a 55 year old man with gastrointestinal NF which ultimately developed MPNST and deceased.