Abstract
INTRODUCTION: Ewing sarcoma (ES) is an aggressive primary bone cancer, primarily affecting children and adolescents, accounting for 5% of cancers in this demographic. ES most commonly affects males during adolescence. It primarily targets flat and long bones such as the pelvis, femur, tibia, and fibula, and less frequently, the mandible and maxilla. CASE PRESENTATION: This case report details a 14-year-old Arab female with maxillary ES, highlighting diagnostic and therapeutic challenges. The patient presented with jaw pain and swelling, initially evaluated via radiography and biopsy, revealing classic histopathological features (small round blue cells, Homer-Wright rosettes) and immunohistochemical markers (CD99, NKX2.2, FLI1 positivity). Imaging identified a localized maxillary mass (T4a N0 M0). Multimodal therapy included neoadjuvant chemotherapy (VDC/IE regimen), en bloc resection, adjuvant chemotherapy, and dental rehabilitation. CONCLUSION: Craniofacial ES often mimics benign odontogenic lesions, delaying diagnosis; this case achieved timely intervention within 7 months. Unlike axial ES, jaw tumors require balancing oncologic control with functional preservation, emphasizing conservative surgery and multidisciplinary rehabilitation. The patient's outcome aligns with studies showing superior survival for craniofacial ES (82% 5-year survival) compared to pelvic sites.