Phylloides tumours are unusual neoplasms, accounting for less than 0.5% of breast tumours and approximately 2.5% of fibro-epithelial tumours. They usually present as fast-growing, painless masses, with a high local recurrence rate. Grading and an adequate surgical resection with tumour-free margins are the most important factors predictive of recurrence. The treatment is based on surgery, with poor results reported for chemo- and radiotherapy, but controversy still exists as to whether the best surgical approach consists in radical or conservative procedures, depending upon tumour size at diagnosis. The authors report the case of a patient affected by a metachronous bilateral malignant phylloid tumour of the breast, involving regional nodes and with a single pulmonary metastasis. The patient was treated with radical surgery including a bilateral mastectomy, an axillary dissection and a right inferior pulmonary lobectomy. Unfortunately, even this substantially aggressive management was unable to change the final outcome of the disease. The review of the literature on the subject is consistent with a preferably conservative surgical treatment also in advanced stages of the disease.