Malignant peripheral nerve sheath tumors (MPNST) comprise 2% of all sarcomas, the median age for sporadic MPNST is between 30 and 60 years, and that for NF1-associated MPNST is between 20 and 40 years. Half of MPNSTs are associated with neurofibromatosis type 1 (NF1), the autosomal dominant condition that, affecting 1 in 3000 live births, represents the most common human cancer genetic predisposition syndrome. The remaining 10% of MPNSTs arise secondary to previous irradiation and account for about 5% of radiotherapy-induced sarcomas. The most common sites of involvement include the nerve roots and bundles in the extremities and pelvis, particularly the sciatic nerve. In most instances, the size of the mass is greater than 5 cm at presentation, and up to 50% of patients present with metastatic disease, usually to the lung. MPNST usually demonstrate a marked increase in tumor cellularity, pleomorphism, and mitotic activity and show a more organized cellular growth pattern, with less extracellular matrix material. In the setting of localized disease, as is the case with all soft tissue sarcomas, complete surgical extirpation with clear margins is the treatment of choice. Our patient had no history of NF-1, and 1 cm lesion smaller than 5 cm occurred locally in the scalp, an atypical location. It has been surgically removed and has been without recurrence. We report this as a rare case that is different from the typical MPNST.