Leiomyosarcoma and its pleomorphic variant are rare entities in the head and neck region. Since they usually present as slow growing, discrete firm, and non-ulcerated painless mass, they seem to be deceptively benign and are thus misdiagnosed. Histopathological and immunohistochemical studies are the ways of getting a definitive diagnosis. Till date surgery has been the primary treatment but effectiveness of radiotherapy/chemotherapy is still questionable. Here, we describe a case of leiomyosarcoma in mandible along with discussion about the ways of diagnosis, its differentiation with its pleomorphic variant and their managements.
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