Abstract
Undifferentiated pleomorphic sarcoma (UPS) is a rare entity found in the oral cavity which involves the mandible in only 3% of all UPS bone lesions. The correlation of the clinical presentation of the neoplasm with the histopathological and immunohistochemistry findings is very important for appropriate diagnosis. Its incidence in membranous bone like mandible is rare. According to our literature search in the PubMed database, there are only four reported cases from India some of which does not have any immunohistochemistry findings to confirm the diagnosis. We present a rare case of UPS of mandible which was clinically mimicking squamous cell carcinoma. Upon immunohistochemical analysis, we found vimentin, KI67, CD68 positive and desmin, Melan A, LCA, S100 and myogenin negative, which lead us to the diagnosis of UPS.