We report a case of lethal midline granuloma in a 34-year-old male patient. The patient was referred to our hospital because of long-lasting hoarseness. He was treated for granuloma laryngis. After two years nasal obstruction developed followed by ulceration of the hard palate and destruction of part of the nose and the upper lip. A biopsy demonstrated polymorphic infiltrate consisting of small lymphocytes, plasma cells, macrophages, atypical lymphoid cells and eosinophils. Radiotherapy was very effective and led to long-term remission. The different etiological aspects of this syndrome are discussed.