Introduction: Adenoid cystic breast carcinoma is a tumour of extremely rare malignant behavior, belonging to the group of triple negative breast tumors but, unlike these, has a favorable prognosis with high survival if the diagnosis and management are appropriate [1,7,9]. Presentation of Case: A 34 year old female patient presented symptom is a well-circumscribed palpable mass in the right breast with recurrent breast pain that not improve with analgesic or progestin gel. Excision biopsy of a lump in the upper outer quadrant revealed adenoid cystic carcinoma. A right sided lumpectomy was performed, with sentinel node biopsy. Histopathology revealed a fibroadenoma accounting for one mass and adenoid cystic carcinoma in another, confirmed on Periodic acid Schiff (PAS) staining, with a very closely margin. A second lumpectomy was performed with focal adenoid cystic carcinoma. Finally a right side modified radical mastectomy (MRM) was performed. The sentinel node biopsy was negative. There was no evidence of recurrence or distant spread at follow-up 12 months postoperatively. Discussion: Adenoid cystic carcinoma of the breast is a rare tumor, of which very few cases have been described in the literature and which lacks in-depth studies on its diagnosis and management due to its rarity [1,2]. Nowadays, its diagnosis is based on biopsy excision and anatomopathological study. And the management, like the Basal_Like tumors of the breast, is based on surgical excision with a wide margin of safety. However, unlike these, axillary nodal study is not justified, and the use of posterior adjuvant is controversial with various opinions in this regard [1,3].

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