Adenoid-cystic carcinoma of the breast is considered a rare entity with a comparatively favourable prognosis. We report the case of a 58 year old woman and review another 150 cases published to date in the pertinent literature. The clinical course and outcome of 99 patients was evaluated by meta-analysis. Recurrence-free ten-year survival was calculated at 85.1 percent following mastectomy and at 45.7 percent after breast-conserving therapy (p < 0.05). Six of ten local recurrences and 7 of 8 distant metastases occurred 5 years or later after initial treatment. It is concluded that patients with adenoid-cystic carcinoma of the breast should be treated by primary mastectomy and extended follow-up is recommended.