Metaplastic breast cancer (MBC) is a malignancy characterized by the histologic presence of two or more cellular types, commonly a mixture of epithelial and mesenchymal components. MBC is rare relative to invasive ductal carcinoma (IDC), representing less than 1% of all breast cancers. Other than a lower rate of lymph node metastases, MBC tumors display poorer prognostic features relative to IDC. Due to its low incidence and pathological variability, the ideal treatment paradigm for MBC is unknown. Because of its rarity, MBC has been treated as a variant of IDC. Despite similar treatment regimens, however, patients with MBC have worse outcomes. Recent research is focused on biological differences between MBC and IDC and potential novel targets for chemotherapeutic agents. This paper serves as a summation of current literature on approaches to the multidisciplinary treatment of patients with MBC.