Primary neuroendocrine carcinoma of the male breast is an exceptionally rare entity, with fewer than 50 cases documented in the literature. We present a case of a 71-year-old male with a rapidly growing retro-areolar mass. The histopathological and immunohistochemical evaluation confirmed a diagnosis of primary neuroendocrine carcinoma of the breast, with positivity for chromogranin A, synaptophysin, and CD56 and a Ki-67 proliferative index of 30%. The patient underwent total mastectomy with axillary lymph node dissection followed by adjuvant tamoxifen. At 24-month follow-up, he remains disease-free. This case highlights the diagnostic challenges of male neuroendocrine breast carcinoma (NEBC) and emphasizes the critical role of histopathology and immunohistochemistry in achieving an accurate diagnosis. We provide a comprehensive review of the literature, propose a management algorithm, and suggest future research directions to address knowledge gaps in this rare malignancy. This report adheres to the SCARE 2020 criteria for surgical case reports.
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