Extranodal natural killer/T-cell lymphoma, nasal type (ENKL) is a rare and aggressive tumor that can mimic inflammatory conditions and other tumors, and present a diagnostic challenge. We report a 57-year-old Chinese man previously misdiagnosed with rhinoscleroma. The patient had undergone multiple biopsies when the histopathological diagnosis of ENKL was made. Because the diagnosis may be obscured by extensive necrosis, multiple biopsies are necessary to make a certain diagnosis. According to the diagnosis process of this patient, the need to consider a neoplastic cause should be highlighted when faced with an aggressive nasal disease not responsive to maximal treatment.