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Background: Skin adnexal tumors (SATs) are rare in Indian subcontinent and hence pose difficulty in diagnosis as they may histopathologically look like more common skin malignancies in this region. Several studies on SATs have been carried out in Western countries but very few in this region.

Malignant myoepithelioma of the breast is a rare lesion characterized by the malignant proliferation of the epithelial and myoepithelial cells that exhibit characteristic histological and immuno-histochemical features. Very few cases have been reported in literature. We report a case of a 64-year-old female with a palpable lump in the upper outer quadrant of her right breast, present for a number of years. She underwent lumpectomy under local anesthesia. Upon histological confirmation of the diagnosis of malignant myoepithelioma, the patient underwent wide local excision with sentinel node mapping. Malignancy was evident by extensive central infarction. The tumour was completely triple negative, i.e. Estrogen receptor (ER), Progesterone receptor (PR) and Human epidermal growth factor receptor 2 (HER2) negative. The patient also underwent adjuvant chemotherapy and radiation therapy. The patient remains well with no recurrence 7 years since her initial surgery. The authors believe, that due to the paucity of these rare tumours, optimal treatment strategies remain to be determined.