Breast cancer in men is an uncommon disease. Because of its rarity little is known about its aetiology, clinical behaviour and treatment. Retrospective studies show that when age- and stage-matched breast cancer in men and women are compared, there is no difference in survival between the two groups. Nevertheless, because of the absence of screening protocols and the limited amount of mammary tissue in men, allowing rapid local infiltration, a late diagnosis is often made, with a poor survival rate. Most of our current knowledge about the biology, natural history, surgical therapeutic strategies, adjuvant radiotherapy and chemotherapy protocols of male breast carcinoma has been extrapolated from its female counterpart. The Authors report the case of a male patient with breast cancer and pagetoid diffusion in the nipple region, and, on the basis of a review of the literature, summarise what is currently known about this rare neoplasm in terms of prognostic factors, therapy and survival.