Pure primary squamous cell carcinoma (SCC) of the breast is a rare condition. The exact histogenesis of this malignancy is unclear. The rarity of the condition makes it difficult to draw firm conclusions on the course of the disease and the overall prognosis. We report a case of pure primary SCC of the breast occurring in a 62-year-old woman and presenting as an enlarged breast lesion with bleeding. We also review the literature for all cases of pure primary SCC.