Desmoid tumor can be defined as a pseudoencapsulated infiltrative growth of well-differentiated collagenous fibroblasts and fibrocytes arising either in fascia or musculoaponeurotic structures. The etiology of desmoid tumors is poorly defined. The most commonly implicated etiologic factors are trauma, hormonal disturbances, and genetic or hereditary factors. Desmoid tumors of the anterior abdominal wall are much less common than extra-abdominal desmoids; they may occur at any age but are most common in the third and fourth decades. Although both sexes may be affected, abdominal desmoids predominate in females, particularly in females of childbearing age. Extra-abdominal desmoids, which most commonly occur on the back, chest wall, head and neck, or lower extremity, have a male predominance. Most patients complain of a painless mass of several months or years' duration. The primary consideration in surgical treatment of desmoid tumors should be the prevention of local recurrence. In most instances, this can be achieved by wide local excision or muscle group resection. There is no clear evidence that irradiation or chemotherapy are effective in controlling desmoid tumors.