A case of nasal T-cell lymphoma as a cause of lethal midline granuloma in a 41-year-old woman is described. Primary chemotherapy as management failed, and tumor control was achieved thereafter by local radiotherapy to a dose of 52 Gy. Fourteen months after diagnosis the patient died in multiorgan failure with involvement of her skin, lung and liver. Present studies give strong evidence that lethal midline granuloma is very often a type of T-cell lymphoma that might be caused by Epstein-Barr virus. According to the literature our findings support the hypothesis that tumors are best treated by local high-dose irradiation.