RESUMO
INTRODUCTION: Primary apocrine sweat gland carcinoma (PASGC) is an extremely rare neoplasia whose management and treatment are still evolving. The only curative therapy is wide local excision. Many patients have metastasis at the time of the diagnosis, mainly because this neoplasm has been misdiagnosed as some benign skin lesions. PRESENTATION OF CASE: We herein report a case of a 72-year-old-man with PASGC affecting the axilla and regional lymph nodes that underwent surgical resection and lymphadenectomy at our Institution. This is the first case reported in Brazil. DISCUSSION: Our observation suggests just a MRI as necessary to study tumoral limits and lymph nodes and a full surgical excision with free margins is decisive for success. CONCLUSION: Despite the PASGC be a rare cancer and require expensive tests, knowledge of this disease is critical to reduce costs in medical services without availability of investment.