Secretory carcinoma of the breast is a rare and indolent tumour originally described in children but occurring equally in the adult population. The principal management problems following primary surgical treatment are local recurrence and axillary lymph node metastases. Distant metastases are extremely rare. We present the case of a 27-year-old woman with pulmonary metastases from a secretory breast cancer treated by mastectomy and axillary lymph node dissection 12 years previously. There was no response to chemotherapy; however, the patient remained alive and active two years from presentation with metastatic disease and one year from cessation of all cytotoxic chemotherapy. She eventually died of respiratory failure two and a half years after presentation. To our knowledge, this is only the fourth reported case of distant metastases from secretory breast cancer and the second reported case in which current active chemotherapy has been used. We review the literature and discuss the apparent chemoresistance of this tumour including the lack of membrane staining for Her2/neu. In the absence of any proven effective chemotherapy we believe that symptom control becomes the focus of management and offers patients with metastatic secretory breast cancer the greatest chance of a functional and good quality existence.