Abstract
Malignant nerve sheath tumors are rare and aggressive soft tissue sarcomas. They contribute to 5-10% of all soft tissue sarcomas. They can be sporadic, occur in patients with NF1 (neurofibromatosis 1) or can occur after radiation therapy. A high rate of recurrence and hematogenous metastasis is seen in these patients. They are also associated with poor prognosis. A case of malignant nerve sheath tumor seen in a 44-year-old male with pre-existing NF1 is being discussed here due to the unique nature of the disease and its rarity.