Abstract. An epidermal inclusion cyst (EIC) of the breast is a rare, benign condition that may potentially be malignant. The present study conducted a systematic review of the literature in order to identify pathological hypotheses, clinical characteristics, and diagnostic and treatment options. A search for relevant studies was conducted through the Scopus, Embase and Medline databases during September 2014. The search term employed was ῾epidermal inclusion cyst breast᾽. Studies were selected if they contained adequate information regarding symptoms at presentation, diagnostic tools, pathology, characteristics, type of procedure performed and follow-up routines. A total of 35 papers describing 91 patients affected by EIC of the breast were identified. Following this, a total of 82 patients, including an additional case supplied from the present study, were selected for further analysis. EIC of the breast typically occurs during the fifth decade of life. A palpable mass of the breast was present in 65 (79%) patients. Ultrasonographic imaging was consistently utilized as a diagnostic tool in all the cases analyzed, whereas fine-needle aspiration cytology was used in 70% of the cases and mammography in 65%. No tumor recurrence was reported at a mean follow-up time of 53 months. The present study demonstrated that elliptical excision is the preferred treatment for EIC of the breast, with pathological analysis required to exclude malignancy.