Background: The prognostic factors and optimal choice of treatment for primary neuroendocrine neoplasms of the breast (BNEN) remain to be defined. Methods: Patients diagnosed with BNEN in China were retrospectively reviewed from the literature following the systematic search of China National Knowledge Infrastructure (CNKI), Chinese biomedical literature service system (sinomed), wanfang medical network, and Pubmed database. The clinical characteristics and different treatment modalities of patients with BNEN were evaluated. Results: A total of 209 cases with BNEN were enrolled. There were 204 female and 5 male patients. The median age was 51 years old (range, 17e82). Out of 209 patients with BNEN, 208 (99.5%) patients were treated with surgery (SG), 44 patients (21.1%) had received radiotherapy (RT), 173 patients (82.8%) experienced chemotherapy (CT). A total of 158 patients with hormone receptor (HR) positive (87.8%, 158/ 180) were treated with endocrine treatment (ET). The median follow-up time was 52.4 months (range, 6 e144). The 3-year overall survival (OS) rate and 3-year disease-free survival (DFS) rate for the whole group were 93.7% and 85.3%, respectively. In univariate analyses, Ki67 expression 20%, HR negative, neuroendocrine carcinomas (NECs) were associated with decreased OS and DFS (P < 0.05). Patients treated with anthracycline/taxane-containing CT regimens, or taxane-containing CT regimens had superior OS and DFS than patients without those (P < 0.05). Among 69 patients with stage I who received CT had no significant differences in OS or DFS compared to those without CT. Multivariate Cox regression analysis showed that gender, HR expression, pathologic subtype, and CT were independent prognostic factors for DFS but not OS (P > 0.05). Conclusions: The best selection of patients to get the most benefit from different treatment modalities warrant further exploration. The clinicopathological parameters including gender, HR expression, ki67 expression, pathologic type, stage, tumor size, and lymph node status may serve as both indicators of diagnosis and prognosis, and guide treatment decisions for BNEN.