One of the leading causes of cancer mortality in women worldwide is breast malignancy. In western countries 3.5-10% of newly diagnosed patients are found to have metastases at diagnosis (MBC). Despite the epidemiological burden of this condition, there are no guidelines on how to manage breast cancer patients presenting with systemic spread; for these patients treatment planning is essentially based on personal preferences rather than reliable clinical data. Through this review we outline the currently available evidence and discuss the role of surgical resection of the primary breast cancer in MBC.