Undifferentiated pleomorphic sarcoma (UPS), formerly known as malignant fibrous histiocytoma (MFH), arises mainly in soft tissues (1). This tumor has been established as an entity in the World Health Organization (WHO) classification of soft tissue sarcoma (STS) since 2002 (2). In general, the occurrence of head and neck sarcoma is rare. The nasal cavity and paranasal sinuses are the most common locations of UPS in head and neck region, and only 0.1 of them appear in the oral cavity, which is very uncommon (3). Although its etiology is still unknown, genetic predisposition, environmental factors such as trauma, history of radiotherapy and the development of malignant tumors from benign lesions have been proposed as its etiology (4). Symptoms usually depend on the involved anatomical site, but the neoplasm usually presents as a rapid growth with or without pain. Due to the high invasive potential of this tumor, the frequency of metastases is very high at the time of initial tumor dia...