Abstract
Ewing's sarcoma (ES) is a small blue round cell tumor, malignant in nature typically affecting long bones and pelvis. Occurrence of ES in the head and neck region is rare and is reported to be 2%-3%, of which the chances of having primary lesion are rare. In the head and neck region, it has been reported to strike skull, supraclavicular region, parotid region, orbital floor, nasal cavity, maxilla, mandible and zygoma. We present a case of primary ES of the mandible in a 22-year-old female who reported with a palpable swelling on the lower left part of the face; and intraorally, there was a growth in the molar region. Orthopantomogram showed multilocular radiolucency in the molar-ramus region. The diagnosis was made after surgical resection, histopathology and immunohistochemistry profiling. The patient was treated by cortical segmental resection of mandible combined with chemotherapy and a follow-up was done for 2 years.