""Midline Granuloma Syndrome"" encompasses a number of specific clinicopathologic entities, such as ""Idiopathic Midline Destructive Disease(IMDD), Polymorphic Reticulosis and Lymphomatoid reticulosis, Wegener's Granulomatosis"" and it manifest variable degree of upper airway destruction accompanied by acute and chronic inflammation with or without granuloma formation. The therapeutic implication as well as the prognosis depend on these histopathologic classification and so repeated and adequate biopsies are required. In the present paper we provide 3 cases of IMDD and 3 cases of Polymorphic Reticulosis to discuss clinicopathologic findings and therapeutic results.