Unlike benign schwannomas, MPNSTs are seldom found in the head or neck area, comprising only 4% of all head and neck sarcomas.[9] MPNSTs in the head and neck (HN-MPNSTs) can be either sporadic or associated with neurofibromatosis type 1 (NF1), an autosomal dominant disorder affecting neurofibromin 1 suppressor gene.[3,10] It was reported that 20% MPNST patients were accompanied with neurofibromatosis and MPNST occurred in 40% to 50% of patients with neurofibromatosis,[6] but our case did not find any signs or symptoms of neurofibromatosis. The gene discussed is NF1; the disease is neurofibromatosis.