Adenocarcinoma of the rete testis is an extremely rare and aggressive tumor that carries a poor prognosis. Successful long-term treatment for such tumors remains elusive as more cases are discovered worldwide. Treatment typically involves radical orchiectomy, retroperitoneal pelvic lymph node dissection, adjuvant chemotherapy, and/or continued surveillance. Here we describe the case of a 42-year-old male with a history of low testosterone who presented with a localized adenocarcinoma of the left rete testis. He was treated with radical orchiectomy and continued surveillance alone due to a lack of evidence of metastasis on follow-up imaging. History, prognosis, diagnostics, and treatment guidelines, as well as the most significant recent cases since the last rete testis adenocarcinoma literature meta-analysis, are discussed.

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