Abstract
INTRODUCTION: Ewing sarcoma of the skull (EWS) is an exceptionally rare variant, accounting for approximately 1% of all Ewing sarcoma cases. Due to its rarity, there are only a limited number of documented instances in medical literature, making it a topic of significant interest and importance in the field of oncology. CASE REPORT: A 13-year-old boy presented with a gradually enlarging swelling measuring 5 × 4 cm in the left parietotemporal region. Histopathological analysis confirmed a diagnosis of Ewing sarcoma of the cranium. The patient underwent neoadjuvant chemotherapy and radiotherapy, followed by surgical resection. However, 2 years later, he experienced a recurrence characterized by exophytic growth and intracranial involvement. Although a revised course of chemoradiotherapy was planned, the patient succumbed to his condition on the 14th day of hospitalization. CONCLUSION: The definitive diagnosis in such cases is often complex and relies heavily on histopathological findings. Early detection, along with prompt multidisciplinary intervention, is essential for effective management and improved patient outcomes.