Ewing sarcoma is a highly malignant neoplasm, which very rarely involves the head and neck region. Only a few cases have been reported in the maxila or palate. This case is being reported for its rarity. A patient presented to the Department of Oral Pathology, Faculty of Dental Sciences, CSM Medical University, Lucknow, India, with a lesion expanding the nasal cavity and posteriorly reaching up to the anterior border of nasopharynx and inferiorly causing the destruction of the hard palate extending into the oral cavity. The biopsy specimen histopathologically revealed undifferentiated malignant neoplasm, and the definitive diagnosis of Ewing sarcoma was made with immunohistochemistry. The patient underwent surgical excision of the lesion followed by radiotherapy and chemotherapy. Schlagwörter: CD99, Ewing sarcoma, malignant tumor, maxilla