We report a case of malignant midfacial granuloma or idiopathic midfacial granulomatosis in a 50-year-old male farmer with no previous history. He was admitted to the department in an emergency context. The history was reportedly 18 months old. He presented a symptomatology made of nasal obstruction, fever, headache and rhinorrhea with an altered general state. He consulted several health facilities. The evolution was marked by the aggravation of the symptomatology. The examination at admission noted an alteration of the general state. Examination of the ENT sphere revealed ulcerative-necrotic lesions with a destructive nasal origin, with bone sequestration, midface edema, and extension to the sinuses and hard palate. The CT scan revealed bony and cartilaginous lysis, irregular thickening of the nasal pyramid, continuity of the nasal septum, and isodensity of the maxillary, ethmoidal, and left sphenoidal sinus walls without total filling. Deep biopsies were performed under general anesthesia. Histology was consistent with a granulomatous reaction with progressive tissue destruction. Mycobacterium tuberculosis was absent. Medical treatment was initiated. The evolution was marked by the extension of the lesions, malnutrition and the appearance of generalized oedema. The patient died on the 45th day of his hospitalization in a septic shock situation. Conclusion: Mediofacial granulomatosis is a difficult lesion to diagnose and manage. It requires careful histological exploration in order to differentiate it from other mediofacial destructive lesions. The evolution is most often towards extension and lethal complications.