Inflammatory breast cancer (IBC) is an aggressive and unique breast cancer diagnosis. Patients tend to have poorer prognosis with increased risks of recurrence and higher mortality rates compared to other types of breast cancer. Additionally, they have a high likelihood of stage IV disease at diagnosis. IBC is defined by diffuse redness and swelling of the skin occupying at least one-third of the breast (so-called “peau d’orange”) in combination with a breast biopsy showing breast carcinoma. The peau d’orange appearance is thought to be due to tumor emboli obstructing dermal lymphatics, resulting in cutaneous edema. A good history and physical exam are important since this is a largely clinical diagnosis and some findings can emulate mastitis. Treatment of stage III IBC consists of multimodal therapy, with neoadjuvant systemic therapy followed by a modified radical mastectomy, then adjuvant radiotherapy and systemic therapy. Immediate breast reconstruction at the time of cancer surgery is not recommended due to the high risk of recurrence. Systemic therapy alone without surgery is recommended for patients with stage IV IBC.