Phyllodes tumor of the breast (PTB) is a type of breast tumor with low clinical incidence. PTB most often manifest as a progressively enlarged painless mass and may easily be confused with other diseases, such as breast fibroadenoma, breast metaplastic carcinoma. Its accurate diagnosis is difficult to achieve by preoperative aspiration biopsy, and the definitive diagnosis is based on molecular pathology after total tumor resection. At present, surgical operation is the main method for the treatment of PTB and extended resection of the tumor for ensuring an adequate negative margin is considered to be the standard surgical procedure. Radiotherapy can further reduce the risk of local recurrence in patients with borderline and malignant PTB. Currently, Studies related to systemic treatments such as chemotherapy are scarce, and the clinical benefit is limited and controversial. Here, the authors review the latest research progress in the epidemiology, clinical presentation, diagnostic imaging, and pathology of PTB at home and abroad, and sort out different new viewpoints of clinical diagnosis and treatment.