Abstract
Ewing sarcoma is a rare, highly aggressive small round cell tumor that primarily affects the bones of adolescents and young adults, with extra skeletal involvement being particularly uncommon. We present the case of an 18-year-old male with a progressively enlarging, painful swelling in the right posterior neck, ultimately diagnosed as Extra skeletal Ewing sarcoma involving the occipital bone and C1 vertebra. Imaging revealed extensive intracranial, intraspinal, and paravertebral extension with multiple skeletal metastases. Histopathological and immunohistochemical analysis confirmed the diagnosis, showing NKX 2.2 positivity. The patient was initiated on systemic chemotherapy using the VDC/IE regimen, leading to significant clinical and radiological improvement. This case underscores the importance of considering Ewing sarcoma in atypical paraspinal and cranial presentations and highlights the critical role of early multimodal imaging and chemotherapy in managing aggressive disease.