Objective To study the clinical features, diagnosis and treatment of extraskeletal Ewing’s sarcoma (EES).Methods The clinical data of a patient with EES were analyzed, and the relevant literatures were reviewed and discussed.Results The clinical manifestations and imaging characteristics of the patient with EES lacked specificity. The diagnosis of EES in this case was dependent on postoperative examination, in which the immunohistochemical results were PAS (+), CD99 (+) and Vimentin (+), and the translocation of EWSR1 gene was detected by gene test. Optimal treatment consisted of surgery in combination with chemotherapy.Relapse occurred after 8 months of the follow-up.Conclusions EES in the neck is extremely rare, which has the parameter of strong invasion and highly malignant.Early diagnosis and comprehensive treatment should be emphasized, but the prognosis were worse.