Ewing’s Sarcoma is type of malignant round cell neoplasm, representing only 1–4% of all ES cases. Temporal bone ES is often confused with chronic otitis media as clinical picture is quite similar i.e., localized swelling, hearing loss, facial palsy and ear discharge.

Diagnosis is by histopathological examination. Treated via surgical excision and chemo radiation. The present article presents a rare case of ES of temporal bone in an eight-year-old female patient .