Introduction: Apocrine carcinoma of breast is a rare type of malignant tumor, the incidence of which varies between 0.3 - 0.4 % of all female breast cancers.Apocrine carcinoma is exceptional in male patients and very few cases have been described in literature. This tumor shows distinct microscopic and immunohistological features. We report an exceptional observation of apocrine carcinoma of breast in a man. Patient and observation: He’s a 54 years old man who had for 2months a painless nodule at the left axillary..The patient had family history of breast and prostate cancers. Physical examination revealed a left axilary lymphadenopathy movable relative to superficial and deep plans with no evidence mass of breasts. MRI of the breast was performed and revealed a mass that was 38 x 10 mm in size.A biopsy of the lymphadenopathy was performed. It objectified a carcinomatous proliferation. An immunohistochemical study showed that tumor cells express Her 2, but do not express estrogenic and progesterone receptors. A tumorectomy of the left breast was performed and didn’t show any malignant lesion of the breast. The axiler dissection of 13 lymphnodes showed 11 metastatics ones with 3 breaking capsular. The diagnosis of apocrine carcinoma of the breast was made in despite of the result of the tumorectomy. The CT did not indicate metastasis. The patient was administered adjuvant chemotherapy then he received radiation therapy on left susclavicular, axila and breast with a total dose of 42Gy,15 fractions of 2.8 Gy on 21 days with no late effects. 1 year of trastuzumab was administrated. There was no recurrence or metastasis approximately 2 years after radiation therapy. Then the patient presented a susclavicular lymphnode that was comfirmed on the pet-scanner with multiple mediastinal lymphnodes. A biopsy of the susclavicular lymphnode comfirmed the progression of the disease. The patient started chemotherapy in association with pertuzumab and trastuzumab. Discussion: Apocrine carcinoma of the breast is a rare malignant tumor whose incidence varies between 0.3% and 4% of all female’s breast cancer and represents 0.5 % of all invasive breast cancers. This tumor is exceptional in men. Indeed, only a dozen cases have been described in the literature . Most neoplasms are slowly progressive, small in size, and are most frequently seen in the axilla. They can be recurrent and metastasize to the lymph node, lung, and bone. Male patients have been advanced disease at presentation compared to women which may be due to lack of public awareness of breast cancer in male. Histologically, it has glandular structures with apocrine features and decapitation secretions. There is cytoplasmic PAS positivity of the tumor cells. The presence of neoplastic glands high in the dermis and immediate subepidermis favors the primary origin of tumor cells from apocrine sweat glands. Apocrine adenocarcinomas are positive for cytokeratins, carcinoembryogenic antigen (CEA) and epithelial membrane antigen (EMA) . Usually, these tumors do not express the estrogen receptor-alpha, progesterone receptors and bcl-2. Apocrine adenocarcinoma has poor prognosis and the prognostic factors include size, histological type, lymph node involvement, and distant metastasis. The 10-year disease free survival rate in the absence of metastasis to the lymph nodes is 56%. Treatment protocols of apocrine carcinoma are similar to non apocrine carcinoma of breast. However studies involving the use of anti androgens are in progress. The treatment of choice is wide local excision with clear margins, with or without regional lymph node dissection. The role of radiation therapy also remains uncertain in the absence of clinical trials. No clear correlation between treatment modality and recurrence in apocrine carcinoma was apparent, and survival rates of apocrine carcinoma were not different from other breast carcinomas. Conclusion: In conclusion, male apocrine carcinoma is a very rare, unique and morphologically-distinctive, invasive ductal carcinoma.. Although immunohistochemical staining might show differences in males, the prognosis is not different from other breast carcinomas. It has different hormonal profile, androgen receptor positivity makes patient with apocrine carcinoma eligible for targeted therapy.