Breast cancer management has evolved from the conventional radical Halsted to a fully integrated multidisciplinary approach. Nipple sparing mastectomy (NSM) is an innovative technique that preserves native breast envelope without mutilation of nipple-areola complex (NAC), thus providing a reasonable alternative for early cancers without NAC involvement and prophylactic high risk patients and avoids multiple surgical procedures required for reconstruction. This article aims to critically review indications, intra-operative protocols, radiotherapy planning and limitations of NSM. Patient selection should be based on study of breast duct anatomy by Magnetic Resonance Imaging, mammographic tumor-nipple distance and obligatory intra-operative frozen section from retro-areolar tissue. Tumor size, axillary lymph node status, lymphovascular invasion and/or degree of intraductal component are factors used to include NSM candidates based on institutional practice. Given the heterogeneity of patients and lack of standardization of preoperative investigations, surgical technique and pathologic sampling of retro-areola tissue so far, mandates a multi-institutional prospective study to define and validate a role for NSM in invasive breast cancer and DCIS. Nipple necrosis is an important NSM complication which can be greatly reduced using alternative skin incisions. Even if the nipple survives, an insensate nipple and lack of sexual function is common and requires preoperative counseling and discussion. Finally the relation and timing of intra-operative versus adjuvant breast radiation and tailoring of dosage and delivery methods has not been fully explored. Although NSM reduces psychological trauma associated with nipple loss, the oncologic safety as well as functional and aesthetic outcomes needs additional investigation. Clin. Anat. 26:56–65, 2013. © 2012 Wiley Periodicals, Inc.