Abstract: The authors show sevem cases of malignant midline granuloma of the Clinic of Otolaryngology of the "Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo "occured in the last fifteen years. They do a literature revision of the pathological aná clinical aspects of this entity. The actual Therapeutics efforts are being considered due to give to the otolaryngologyst, in his dayly practice, the more importante aspects in the management of these patients.