| Summary: | Introduction Most seminal vesicle malignancies are secondary to prostate or bladder cancer. Herein, we report a case of primary clear cell carcinoma of the seminal vesicle. Case presentation A 27‐year‐old man was referred to our department for hematospermia and macroscopic hematuria. A digital rectal examination showed a soft elastic prostatic mass. Cystoscopy showed no bladder abnormalities, and tumor marker tests were unremarkable. Contrast‐enhanced computed tomography and magnetic resonance imaging revealed a cystic tumor containing an enhanced nodule near the prostate and seminal vesicle. The tumor was removed en bloc with the prostate and seminal vesicle through a laparoscopic radical prostatectomy. A histopathologic examination confirmed the diagnosis, with the tumor likely arising from a remnant Müllerian epithelium. A 1‐year follow‐up revealed local tumor recurrence, prompting laparoscopy. Conclusion A standard therapy for primary seminal vesicle carcinoma has not been established. Further studies are necessary to determine the optimal treatment strategy.
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