Primitive neuroectodermal tumor/Ewing's sarcoma (EWS) is a very rare tumor in the nasal cavity. It can be easily confused with sinusitis, polyps and other diseases. The most common symptoms are nasal congestion, epistaxis, olfactory disorders and facial pain. In our case, there was a huge tumor developed in the left nasal cavity and invaded orbita detected in physical examination. The diagnosis was EWS and the patient was given chemotherapy and radiotherapy. A rare nasal cavity tumor, EWS, is presented in this paper.