To investigate the clinicopathological features, diagnosis, treatment and prognosis of Myoepithelial carcinoma arising in adenomyoepithelioma (MECAME), and reviewed the literature. The patient was a 46-year-old female with an about 4 cm mass in the right breast. The morphological characteristics were atypical cells “cuff like” surrounded the mammary ductal epithelium with myoepithelial cell immunophenotype. Modified radical mastectomy was performed, and TC chemotherapy was given 3 times after diagnosis. The patient was followed up for 18 months without recurrence and distant metastasis. MECAME is a rare malignant tumor of breast with characteristic morphology and immunophenotype.