Ewing sarcoma (ES) of the head and Neck is a rare entity. The most common location for ES is long bones accounting for 58%, with head and neck accounting only for 3% of all the sites. Here we highlight a unique presentation of ES involving the maxillary region in a 21-year-old male. Patient presented with right maxillary swelling for the last 2 months. Imaging studies reveal osteolytic lesion involving the right maxilla. Ewing's sarcoma rarely affects the head and neck region, posing diagnostic challenges as it mimics many common lesions. Accurate diagnosis requires a multidisciplinary approach involving clinical, radiological, histopathological, and molecular evaluations.