Abstract Adenocarcinoma of the rete testis is a rare neoplasm with 40 reported cases in the world literature to our knowledge. Although the natural history of this tumor is not well known, it appears highly malignant even in localized forms. Adenocarcinoma of the rete testis is highly resistant to adjuvant radiotherapy and any known chemotherapeutic regimen. We recommend radical orchiectomy followed by a retroperitoneal lymphadenectomy as the treatment for clinically localized Stage A tumors and minimal or microscopic Stage B disease. We report a case of adenocarcinoma of the rete testis and review the world literature.