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Desmoid tumors are benign tumors that can be locally agressive. These tumors are infrequent. The incidence is 2.4 to 4.3 % cases per million in general population. Little is known about the true pathophysiology of these tumors. Hormonal, genetic, trauma have been reported for occurence of desmoid tumors. But most commonly desmoid tumors are idiopathic. They may be associated with Gardner syndrome and mutations of the familial adenomatous polyposis (FAP) gene. Spontaneous desmoid tumors most commonly are related with beta-catenin gene mutations. This beta-catenin defect causes activation of Wnt/catenin signaling. Desmoid tumors of head and neck region consist of 7-15% of all desmoid cases. The localizations of the tumor is variable. The behaviour and clinical course can not be estimated.For these characteristics there is no standard consensus on treatment approach. Treatment of desmoid tumors based on surgical. The aim of surgery is to maintain low recurrence and morbidity.We wanted to remind the etiology,pathophysiology and treatment modalities under guidance of the literature.