Malignant peripheral nerve sheath tumor (MPNST) is defined as spindle cell sarcomas arising from or differentiating towards the cells of the peripheral nerve sheath. MPNST is extremely rare in the parotid gland and, in most cases, the prognosis is grim. It usually develops in the setting of Neurofibromatosis type-1 (NF1), but can also develop sporadically. Herein, we report a rare case of MPNST of the parotid gland, in a patient with no previous history of NF1. rnrnA 45 years old patient presented with a non-tender right parotid swelling of one-year duration. A sudden increase in the size of the parotid mass over the past two months alerted the surgeon to proceed to investigate the nature of the mass and the etiology of the sudden change in size. The patient’s initial investigations, which included ultrasound imaging of the swelling, fine needle aspiration (FNA), Computed Tomography (CT) scan, and Magnetic Resonance Imaging (MRI) of the neck and swelling were consistent with a benign pleomorphic adenoma of the parotid gland. However, during the surgery the dissection was difficult and the tumor appeared adherent to the surrounding tissue, which lead to a total parotidectomy, in piecemeal! rnrnThe histopathology revealed a low-grade spindle cell neoplasm exhibiting a diffuse, infiltrative growth pattern, invading into and around salivary lobules, adipose tissue, skeletal muscle bundles and fascia. The morphology and positive s100 & CD34 stains suggested diffuse neurofibroma. Scattered foci of epithelioid cells with increased pleomorphism and mitotic activity were also evident.rnrnThe diagnosis rendered was thus MPNST of the parotid gland arising from a diffuse neurofibroma. The patient was also diagnosed with NF1 based on clinicopathologic correlation with a referral for confirmatory genetic testing. The patient underwent radiotherapy to continue his treatment following the resection of the tumor. rn