A 78-year-old retired woman was diagnosed with metaplastic breast carcinoma (MBC), a rare tumor, in our hospital. We reviewed 15 articles with a total of 1328 patients to determine the epidemiology, clinical features, biomarkers, histology, management and outcome of patients with this tumor. The mean age at presentation is 58.5 years (range 32-83). Eighty-one percent of patients presented either with a breast mass or abnormal mammographic finding. Twenty-three percent of patients had a family history of breast cancer. Estrogen receptors were only found in 12%, progesterone receptors in 10% and HER2 in 6% of patients. The main method of treatment was mastectomy (66.9%) in combination with chemotherapy (57%) and radiotherapy (47%). Five-year disease-free survival ranged between 40% and 84% and 5-year overall survival ranged between 64 and 83%. We have further reviewed the nature of this disease in the light of advancement in genetics, such as microarray gene expression profiling. The relationship of MBC with triple-negative tumor and basal-like tumor is discussed. It is hoped that advances in genetics and biomarkers will bring forward the era of personalized medicine in the treatment of breast carcinoma.