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Desmoid tumor of the breast is an extremely rare entity. Histologically, it corresponds to a benign fibroblastic proliferation of a local evolution, with a high recurrence potential. We report the first case of elderly woman presented with desmoid-tumor of the breast as a manifestation of Gardner’s syndrome. A brief literature review was provided. We report the case of a 72-year-old woman who presented with five months history of painful and an exophytic mass of her left breast. She is known to have familial polyposis and had a total colectomy with Gardner’s syndrome. She had a history of osteomas of the maxilla. On clinical examination, there was an exophytic painless mass, on the upper medial quadrant of the breast. She had also lipoma in the left leg and pigmented skin lesions in legs. Her mammograms showed a suspicious stellar image. A computed tomography scan showed an ovoid lesion. Excision of the lesion was performed. Histopathology confirms a desmoid-tumor of the breast. Evolution was marked by lesion recurrence. In conclusion, desmoid tumor of the breast is rare, non-metastatic but locally aggressive. Clinical expression is often nonspecific. Treatment remains controversial; surgical excision is the treatment of choice.