Ewing sarcoma (ES) is an uncommon malignancy arising most frequently in the long bones of the extremities and the pelvis. Primary Ewing tumors of the head-and-neck region are extremely rare, making up 1% to 9% of all Ewing tumors. Because ES typically affect children and young adults, differentiation from benign congenital neck mass were paramount. The authors experienced a case of ES in a 4-year-old boy, which was manifested as isolated midline neck mass mimicking thyroglossal duct anomaly. We report the case with a brief review of the literature.