Granular cell tumor (GCT) of the breast is an uncommon benign lesion that is grossly and microscopically similar to mammary invasive carcinoma. Thus, it is highly possilbe to misdiagnose this benigh lesion as a malignancy on rapid frozen sections. Herein, we report a case of a 61-year-old woman with GCT of the breast. Immunohistochemically, tumor cells demonstrated positive immunoreactivity for S-100 protein, but showed negative immunostaining for neuron specific enolase (NSE), carcinoembryonic antigen (CEA) and estrogenic receptor (ER). Ultrastructural observations revealed numerous lysosomes bodies and scattered angulate bodies in the cytoplasm of these tumor cells. The basal lamina was also found occasionally surrounding some of these tumor cells. These findings suggest that the GCT might likely have arisen from neural crest-derived peripheral nerve-related cells. The literatures would be reviewed and discussed.