Granular Cell Tumors (GCTs) or Abrikossoffs tumors are ubiquitous lesions that are currently assumed to arise from perineural Schwan cells. Granular Cell Tumors of the Breast (GCTBs) are rare tumors, representing about 5 to 15 % of all GCTs and 6,7 out of 1000 cases of breast neoplasms. They occur usually in premenopausal African-American women and are by large benign, even though in extremely rare cases they can express malignant behavior or coexist with malignant lesions. GCTBs are of particular significance as they mimic breast malignancies both clinically and radiologically. Histological examination is mandatory for the diagnosis of GCTBs and IHC seems to be the gold standard for this diagnosis, indeed, our tumor cells show strong positivity for the S-100 protein. Wide local excision is the only treatment for GCTBs that are associated with an excellent prognosis. In this article we report a case of GCTB with a brief literature review, the aim of this work is to draw the attention of senologists and pathologists toward this misleading tumor, wich should be routinely recalled as differential diagnosis of breast malignancies in order to avoid over treatement of patients.