Cases of coincidental phyllodes tumor (PT) of the breast and invasive breast cancer are extremely rare. This case report describes a 58-year-old women with simultaneous bilateral invasive breast cancer. On the left side, the carcinoma was associated with the epithelial component of a PT and presented with lymph node metastasis. To the best of the present authors’ knowledge, this is the first report of a PT with simultaneous bilateral breast cancer and equilateral lymph node metastasis. Moreover, associated axillary lymph node metastasis with an invasive ductal carcinoma (IDC) arising from the epithelial component of a PT is extremely rare. In the absence of standardized treatment option, therapy recommendations should be tailored individually upon clinical presentation.