OBJECTIVES: Lethal midline granuloma is a syndrome characterized by progressive destruction of the nasal cavity, paranasal sinuses, palate, and deep tissues of the mediofacial region. Lethal midline granulomas are classified as Wegener's granulomatosis, polymorphic reticulosis, non-Hodgkin's lymphoma, or idiopathic midline granuloma. Non-Hodgkin's lymphoma of the nasal type is a rare disease that occurs around the 4th decade and predominantly affects men. It is strongly associated with infection by the Epstein-Barr virus (EBV) resulting in the migration of natural killer cells. We aimed to report a case of this disease in a young patient, in the context of previously published data. CASE REPORT: A 23-year-old man presented at our institution 5 months ago with odynophagia, evening fever, dry cough, and weight loss. Over time, the cough had become associated with runny brown, foul-smelling discharge, worsening of the patient's general condition and epistaxis. On physical examination, we noted a very emaciated patient with necrosis of the nasal cavity and mutilation of the left tonsil and tonsillar pillars. The patient was administered broad-spectrum antibiotics, blood samples were obtained for analysis, and the nasal cavity and pharynx were biopsied. The cultures were EBV positive. Histopathological analysis revealed extranodal lymphoma (nasal type). The patient's condition progressed rapidly, destroying the palate and nasal cavity. The patient was subjected to surgical debridement, clinical monitoring, and radiotherapy with chemotherapy. CONCLUSION: Midline granuloma is a disease that is extremely aggressive, difficult to diagnose, and associated with a poor prognosis. Awareness of this condition at the time of diagnosis will result in more tailored treatment strategies for affected patients.