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Introduction Adenocarcinoma of the rete testis is a rare malignancy with a poor prognosis. We report a case of adenocarcinoma of the rete testis with a durable response to cisplatin‐based chemotherapy. Case presentation A 48‐year‐old man with Down syndrome (trisomy 21) presented with a 1‐month history of painless swelling of the left scrotum. The physical examination revealed a left testis with a hydrocele associated with a tumor and enlarged pelvic and para‐aortic lymph nodes. He underwent a radical orchiectomy. The specimen was diagnosed as adenocarcinoma of the rete testis. The patient received 7 cycles of chemotherapy (1 cycle of BEP and 6 cycles of EP) postoperatively. The metastatic lymph nodes were reduced in size for at least 12 months. Our patient with adenocarcinoma of the rete testis obtained an acceptable response to cisplatin‐based chemotherapy. Conclusion We treated a patient with an adenocarcinoma of the rete testis who had an acceptable response to platinum‐based chemotherapy.