We report a case of endometrioid adenocarcinoma of the prostate. A 60-year-old man visited our hospital with high serum prostate specific antigen (PSA) level (6.4 ng/ml). Digital rectal examination revealed prostatic enlargement but no induration was palpable. Magnetic resonance imaging (MRI) of the prostate demonstrated the low intensity mass lesion around the prostatic urethra on T2-weighted images. Because a prostate cancer revealed by transrectal and transperineal needle biopsy of the prostate, retropubic radical prostatectomy was performed and histological finding showed endometrioid adenocarcinoma of the prostate. We review 41 cases reported in the Japanese literature and discuss them briefly.

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