Ewing's sarcoma (ES) is a small round cell tumor that mainly involves the skeleton, with an incidence of 2.93 per million. It is responsible for 4-10% of all bone tumors, with long bones and pelvis being the most frequently observed locations, whereas it affects the skull in only about 1-4% of cases. After osteosarcoma, ES is the most frequently observed bony primary malignant tumour in children. It more frequently regards adolescents or young adults. It is rarely diagnosed below 5 years of age or after the age of 30. ES rarely occurs in the head and neck district and, when it occurs, the jaw and skull base are the two most frequently involved locations. Clinically, head and neck ES does not show any pathognomonic finding, with the most frequent signs and symptoms usually due to the mass effect of the neoplasm. The aim of this paradigmatic case report is to present and discuss the diagnosis and management of a rare case of ES of the right pterygo-palatine region in a 5-year-old girl, that had been previously misdiagnosed and managed as a dental abscess.