Granuloma gangraenescens or "midline granuloma" (Stewart's malignant granuloma, idiopathic midline destructive syndrome) is a rare clinical syndrome. In clinical practice, the destructive process of the facial midline may appear as a symptom of various infective, malignant or autoimmune diseases. In addition to these cases, a number of cases have been reported in the literature since 1896 with the diagnosis of lethal midline granuloma. These subjects underwent a progressive and often fatal destructive process of unknown cause which generally initiated in a nasal cavity (or sometimes on the palate) in the form of a granulomatous lesion with a rapid tendency to necrosis. The lesion affects the nose, paranasal sinuses, palate and soft facial tissue. As it progresses, it involves soft tissue, cartilage and the bone structures of the aforesaid and adjacent areas. A number of systemic symptoms may be present. The age of patients ranges from a minimum of 15 to a maximum of 59 years old. Repeated biopsies are usually necessary before the syndrome is diagnosed. From a histological point of view, it has been reported that the tissues in the nasal cavities are affected by diffuse infiltration of lymphocytes and leukocytes and histiocytic-type cells. The disease lasts between 3 and 20 months and causes death due to secondary infection, hemorrhage caused by the erosion of major vessels in the head and neck, and cachexia. With regard to therapy, the majority of authors appear to agree that local high-dose radiations in the area of the lesion are the best method of treatment for this pathology.(ABSTRACT TRUNCATED AT 250 WORDS)