Midline granuloma is a mutilating process produced by a number of diseases that progressively destroy the nose, paranasal sinuses, and palate. Infectious, neoplastic, and idiopathic forms of this disease have been described. The specific diagnoses must be ascertained, as the treatment is different depending on the etiology of the disease. Radiation therapy is the treatment of choice for idiopathic midline destructive disease, while cytoxan is appropriate for Wegener's granulomatosis, polymorphic reticulosis, and primary nasal lymphomas. When the diagnosis is uncertain, the least-toxic therapy should be used. If the treatment is failing, an alternate therapy should be tried. This article reviews the history of idiopathic midline granuloma, describes the current classification of the disease, and discusses controversial issues demonstrated by two patient presentations.