We present a case of a 38-year-old male patient with a left axillary metastatic mass without evidence of the primary lesion. Needle aspiration biopsy was performed showing a HER-2 positive occult breast cancer (OBC). The patient underwent neoadjuvant chemotherapy and radical modified mastectomy followed by radiotherapy and systemic therapy. Genetic studies were negative, and the follow-up showed no relapse. OBC is defined as a mammary gland neoplasm that presents as a metastatic disease without clinical or radiological manifestation of the primary tumor. The incidence of OBC in men is unknown and is limited to case reports. A systematic review was performed including eleven case reports with twelve patients ranging from 29 to 83 years old. Estrogen and progesterone receptors were positive in seven patients of which two patients were also HER-2 positive; one patient presented only progesterone receptors, two presented only HER-2 positive and two were triple-negative. Operative management, hormonal therapy, chemotherapy, and radiotherapy were performed together or apart depending on the attending and the patient's preferences.No one reported mortality due to malignant disease. OBC is a rare pathology in men and requires a high diagnostic suspicion in patients with an axillary or thoracic metastatic disease without an evident primary neoplasm. The genetic study is necessary to determine mutations and evaluate the risk of relapse and the high risk of primarily associated neoplasm in the patient and family.