Background Adenomyoepithelioma (AME) is a rare type of benign breast tumor. Many AMEs have demonstrated benign behavior, but very few cases of malignant AME have been reported. Case presentation A 53-year-old woman visited our hospital with a 16-mm, palpable mass in the right breast. Core needle biopsy was performed. The pathological diagnosis was AME. Partial mastectomy was performed. Two years later, local recurrence developed, and the patient again underwent partial mastectomy. The pathology showed malignant AME, and the margin was negative. Eight months later, local recurrence developed again in the same location, and total mastectomy was performed. The pathological diagnosis was malignant AME. The patient has remained recurrence-free without adjuvant therapy.Conclusions A very rare case of AME with repeated local recurrences and malignant transformation was reported.