Inflammatory breast cancer (IBC) is a rare but often fatal disease. This review discusses the following conclusions: (1) The diagnosis of IBC is based on the clinical triad of erythema, ridging with peau d'orange, and rapid onset. The importance of histologic evidence of dermal lymphatic involvement is controversial. (2) Combining doxorubicin-containing chemotherapy with mastectomy or radiation therapy improves survival over that achieved with mastectomy or irradiation alone. (3) Mastectomy after induction chemotherapy may not improve survival or decrease locoregional recurrence rates, but the surgery does provide important prognostic information on treatment response and enables use of a lower radiation dose afterward, which results in reduced long-term complications. (4) The optimal number of cycles and dose intensity of chemotherapy for IBC remain undefined.