Facial mucormycosis and extranodal natural killer/T cell lymphoma (NK/TCL) are characterized by facial progressive swelling, ulceration and destruction. Patient one was a 58-year old woman, who had typical clinical-pathological symptoms of tumor. She was hospitalized in order to remove the tumor. But the diagnosis turned out to be primary mucor infection, which was corrected by positive tissue culture and fungal elements in histology. She was also diagnosed with type 2 diabetes mellitus and acute myelocytic leukemia M2a. With the antifungal therapy, her symptoms, signs and general conditions improved. Regrettably, she gave up the treatment, and died three months later for unknown reason. Patient two was a 60-year-old woman, with the complaints of chronic sinusitis, swelling and necrotic lesion in the right temporal area. Although the mycelium was recovered in biopsy tissues from the necrotic lesion, the diagnosis was revised to extranodal NK/TCL by reviewing the histopathological features and immunophenotypic analysis. The patient also voluntarily abandoned treatment, and died at home for unknown reason. The differential diagnosis of facial mucormycosis and extranodal NK/TCL is usually perplexing. (C) 2016 Elsevier Masson SAS. All rights reserved.