A 38-year-old man was diagnosed with malignant peripheral nerve sheath tumour of the maxilla. He was treated with total maxillectomy. Histopathological examination of the resected specimen revealed a close resection margin. The tumour was of high grade with an MIB-1 labelling index of almost 60%. At six weeks following the surgery, he developed local tumour relapse. The patient succumbed to the disease at five months from the time of diagnosis. The present report underlines the locally aggressive nature of malignant peripheral nerve sheath tumour of the maxilla which necessitates an early therapeutic intervention. A complete resection with clear margins is the most important prognostic factor for malignant peripheral nerve sheath tumour in the head and neck region. Adjuvant radiotherapy may be considered to improve the local control. Future research may demarcate the role of targeted therapy for patients with malignant peripheral nerve sheath tumour. 1. Introduction Malignant peripheral nerve sheath tumours (MPNSTs) encompass a cluster of tumours which arise from the peripheral nerves or display differentiation along various elements of the nerve sheath, namely, Schwann cells, perineural fibroblasts, or fibroblasts [1]. An origin of these tumours in the head and neck region is uncommon occurring in approximately 10% of all cases [2, 3]. Herein, a case of maxillary MPNST is discussed along with the literature review of head and neck MPNSTs. 2. Case Report A 38-year-old man presented with an 8-month history of pain in the left cheek, bleeding from the left nostril, and nasal blockage. He had noticed a swelling over the left cheek and dorsum of the nose which progressed over a period of 6 months. He had also noticed an ulcer on the left upper alveolus which progressed over a period of 2 months. He had a 10-year history of tobacco consumption. A contrast enhanced computed tomography (CECT) of the paranasal sinuses (PNS) was performed which revealed a mass lesion in the left maxillary sinus. He had undergone a surgery for the removal of mass in the left maxillary antrum and nasal cavity by Caldwell Luc approach. Histopathological examination of the resected mass was reported as angiofibroma. After remaining asymptomatic for 5 months, the patient presented with the similar complaints as before for duration of one month. The patient was now evaluated in our multidisciplinary clinic by a team of head and neck surgeon, radiation oncologist, and a medical oncologist. Physical examination revealed swelling of ？cm size over the left cheek involving the nasolabial
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