A 43-yr-old patient visited our department with intermittent chest pain he had suffered with for the past 2 months. Chest CT showed an egg shaped mass in the left chest wall. Local resection was performed for distinguishing the mass from a neurogenic tumor. The diagnosis was periosteal Ewing sarcoma of the rib. Since there was no evidence of metastasis based on the PET-CT, a 2nd operation was done with wide resection and thoracoplasty. The patient was then treated with combined chemotherapy. There has been no local recurrence for the last 1 year. The patient's age and tumor origin were distinct from the usual characteristics of Ewing's sarcoma. Periosteal Ewing's sarcoma of the rib has rarely been reported. We report here on a case of periosteal Ewing's sarcoma of the rib along with a review of the relevant medical literature.