Objective: To study the clinicopathological characteristics, immunophenotype and prognosis of simple apocrine carcinoma (AC) of breast and precancerous lesion. Methods: The medical records and histological diagnosis of 10 patients with simple AC of breast were evaluated retrospectively, and these 10 cases were studied with immunohistochemical staining. Related literatures were also reviewed. Results: The mean age of the 10 cases was 60.5 years old, with 3 cases of apocrine ductal carcinoma in situ (ADCIS), 5 cases of ADCIS with invasive apocrine carcinoma (IAC), 1 case of apocrine encapsulated papillary carcinoma and 1 case of apocrine encapsulated papillary carcinoma with IAC were included. The microscopically characteristics of AC cells in the 10 cases were presented as follows: the enlarged cells with distinct borders, cells with abundant eosinophilic or clear cytoplasm containing granules, high nuclear grade, and large nuclei with prominent nucleoli. Immunohistochemically, the neoplastic cells were positive for androgen receptor (AR), while negative for estrogen receptor (ER) and progesterone receptor (PR) in 10 cases; positive diffuse for gross cystic disease fluid protein 15 (GCDFP-15) in 9 cases; positive for human epidermal growth factor receptor 2 (HER2) in 4 cases. In addition, the histological spectrum of apocrine lesions and precancerous lesions could be found in 10 cases. Clinical follow-up information demonstrated that 9 cases were survival and had no recurrence and metastasis in 12–46 months. Conclusion: Simple AC of breast is a rare specific entity of breast carcinoma, showing unique cell morphology and immunophenotype and often with benign and atypical apocrine lesions. Simple AC of breast may have a well prognosis than an invasive breast carcinoma of no special type. Further studies are needed with regard to its prognosis.