Appropriate surgery in women with retroareolar breast cancer should allow resection of the cancer with wide free margins and an acceptable cosmetic result. The aim of this study was to compare breast conservation surgery (BCS) to mastectomy for treatment of retroareolar breast cancer. In a prospective nonrandomized study, 69 women with retroareolar breast cancers underwent either central quadrantectomy (n=33) with complete removal of the nipple-areola complex or mastectomy (n=36). Two of 33 (6%) patients scheduled for BCS had a secondary mastectomy and immediate reconstruction due to involved margins. After a median follow-up of 42 month (range 17-99 months) in the BCS group and 43 months (range 16-118 months) in the mastectomy group local and regional recurrences as well as systemic disease were comparable between both groups. The postoperative cosmetic result after BCS as evaluated by the patients was rated as excellent in 80% and good in 20% with no poor result. BCS followed by radiation therapy is a feasible alternative to mastectomy in patients with retroareolar breast cancer.