Abstract: Extraskeletal Ewing sarcoma (EES) in nasal cavity is a rare disease, its imaging features, especially those on ¹⁸F-fluorodeoxyglucose (FDG) PET/CT imaging, have been rarely reported in studies. The authors reported the ¹⁸F-FDG PET/CT imaging features of a patient with EES in nasal cavity. The patient in this case presents with a soft tissue mass in the nasal cavity and a significant increase in radioactive uptake. The density and radioactive uptake increased uneven in the bone metastasis site. The authors reviewed the literature to enhance the understanding of the disease. The differential diagnosis of the disease including squamous carcinoma, lymphoma, melanoma, adenoid cystic carcinoma, olfactory neuroblastoma in nasal cavity.