Abstract
Ewing sarcoma is one of the rare, highly malignant neuroectodermal tumors frequently involving bones. Primary orbital Ewing sarcoma is extremely uncommon. We report a rare case of a 5-year-old boy who presented to us with a painless, slow-growing mass above the medial canthus of the left eye, the clinical appearance of which was masquerading as an internal angular dermoid. The child was subsequently diagnosed to have primary orbital Ewing sarcoma. Primary orbital Ewing sarcoma is a rare tumor with poor prognosis, poses diagnostic challenges, and demands a high index of clinical suspicion.