Background: Paget's disease (PD) of the breast is a rare presentation of breast malignancy. Mastectomy has been considered the standard of care, but several studies have shown that breast-conserving surgery (BCS) with radiotherapy is an alternative option. According to the literature, the total recurrence rate is as high as 20-40%. In an effort to enable a rational therapy decision, we compiled and analyzed the available data on PD. Methods: Pubmed, Web of Knowledge, and China Journal Net were searched for studies concerning treatment for PD between 1990 and 2013. A meta-analysis was performed to compare the effect of different treatments. Results: 7 studies with a total of 685 patients were included. The cumulative local recurrence rate was 7.6%, 5.6% among women undergoing mastectomy and 13.2% among those treated with BCS. Compared to the BCS group, mastectomy showed significant differences in terms of local recurrence (odds ratio = 0.38, 95% confidence interval 0.21-0.69; p = 0.001). Conclusion: Since not all currently available diagnostic and therapeutic options (magnetic resonance imaging, radiotherapy, tamoxifen) were systematically used in the studies included in this meta-analysis, it may be presumptuous to suggest that mastectomy is the right choice for all patients with PD in a modern interdisciplinary setting. Rather we can conclude that all efforts should be undertaken to assure local control since BCS is not equivalent to mastectomy in the treatment of PD.